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=Classification=
=Classification=
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===Dextrocardia===
===Dextrocardia===


This term is applied clinically to all cases in which the  
This term is applied clinically to all cases in which the heart is found displaced to the right side of the thorax. Pathologically two conditions must be distinguished . In the one the heart is simply displaced to the right as a result, usually, of acquired disease {De.rtroversio cordis). In the other the organ is also more or less altered in its position in the thorax, so that the apex comes to point to the right. In these latter cases two forms must again be recognized :  
heart is found displaced to the right side of the thorax. Pathologically  
two conditions must be distinguished . In the one the heart is simply  
displaced to the right as a result, usually, of acquired disease {De.rtroversio cordis). In the other the organ is also more or less altered in its  
position in the thorax, so that the apex comes to point to the right. In  
these latter cases two forms must again be recognized :  


:A. The heart is not transposed, but appears to have undergone a simple rotation from left to right on its vertical axis, so that its left chambers come' to lie more' anteriorly and its right chambers more posteriorly. The apex points to the right but is formed not of the left but of the right (venous) ventricle, which remains on the right side and receives blood from the right (venous) auricle into which the venae cavae empty. This, which is not a true transposition, is the condition present in the majority of the cases of congenital dextrocardia. It is well illustrated by Grunmach's^ case (See Fig. 21.)  
:A. The heart is not transposed, but appears to have undergone a simple rotation from left to right on its vertical axis, so that its left chambers come' to lie more' anteriorly and its right chambers more posteriorly. The apex points to the right but is formed not of the left but of the right (venous) ventricle, which remains on the right side and receives blood from the right (venous) auricle into which the venae cavae empty. This, which is not a true transposition, is the condition present in the majority of the cases of congenital dextrocardia. It is well illustrated by Grunmach's^ case (See Fig. 21.)  
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The etiology of these two t^^pes of congenital dextrocardia differs  
The etiology of these two t^^pes of congenital dextrocardia differs widely. Nagel reviews six cases with clinical and autopsy reports in the literature, and demonstrates, with the aid of diagrams, that five of these, namely, those by Grunmach, Geipel, Lowenthal, Barmwerth and himself, belong to the type A above, in which the heart is not transposed, but presents simply a persistence of the embryonic stage, in which the apex was formed by the right half of the common ventricle. The fact that in all five of these cases the grave anomalies of pulmonary stenosis and ventricular septal defect were associated, affords convincing proof that a primary arrest of development had occurred.  
widely. NageP reviews six cases with clinical and autopsy reports in  
the literature, and demonstrates, with the aid of diagrams, that five of  
these, namely, those by Grunmach, Geipel, Lowenthal, Barmwerth and  
himself, belong to the type A above, in which the heart is not transposed, but presents simply a persistence of the embryonic stage, in which  
the apex was formed by the right half of the common ventricle. The  
fact that in all five of these cases the grave anomalies of pulmonary  
stenosis and ventricular septal defect were associated, affords convincing  
proof that a primary arrest of development had occurred.  




In the sixth case, on the other hand, that by Graanboom, in which  
In the sixth case, on the other hand, that by Graanboom, in which there was true transposition of the heart, and the septiun was entire, the probable explanation is to be sought, not in an arrest of development, but, with the causes of transposition in general, in the altered relation of the embryo to the primitive chorionic villi.  
there was true transposition of the heart, and the septiun was entire, the  
probable explanation is to be sought, not in an arrest of development,  
but, with the causes of transposition in general, in the altered relation  
of the embryo to the primitive chorionic villi.  
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[[File:Abbott 21.jpg|600px]]


Fig. 21  
'''Fig. 21''' 1, 2, 3, 4. Diagrams (1) showing normal heart; (2) embryonic heart; (3) mirror picture of normal heart; (4) heart in usual type of pure congenital dextrocardia; (5) from Graanboom's case of pure dextrocardia (mirror picture). Note that the apex is formed by the left (pulmonary blood) ventricle. (From Nagel's article on Pure Congenital Dextrocardia in the Deutsches Archiv. f. klin. Med., xcvi, p. 572.) (6) Grunmach's case of pure congenital dextrocardia; arrest of development, persistent embryonic stage. Note that the apex is formed by the right (caval blood) ventricle. (From his article in the Ben. klin. Wchnschr., 1890, page 22.) The auricles are designated "caval" and "pulmonary" (according to the veins they receive) , instead of right and left, in order to show the difference in situation in the mirror picture and in Graanboom's case, from that of the usual type of pure congenital dextrocardia (Grunmach's jase).  
 
 
1. Normal heart. .-1, caval auricle; B, pulmonary veins auricle; C, right ventricle (caval
blood ventricle) ; D, left ventricle (pulmonary
blood ventricle).
 
2. Embryonic heart. A, caval auricle; B, pulmonary veins auricle; C, right ventricle (caval blood ventricle) ; D, left ventricle (pulmonary blood ventricle).  
 
3. Mirror picture of normal heart. A, caval auricle; B, pulmonarj^ veins auricle; C. right ventricle (caval blood ventricle) ; D, left ventricle (pulmonary blood ventricle).  
 
4. Pure congenital dextrocardia. A, caval auricle; B, pulmonarj' veins auricle; C, caval blood ventricle; D, pulmonary blood ventricle. Note similarity to No. 2.
 
 
5. Graanboom's case of congenital dextrocardia  
(mirror picture). A, caval auricle; B, pulmonary blood auricle; C, caval blood ventricle; D,
pulmonary blood ventricle.
 
 


6. Grunmach's case of pure congenital dextrocardia (arrest in embryonic stage). E, vena cava
superior; F, ventriculum dextrum; G, aorta; H ,
ventriculum sinistrum.


<gallery>
File:Abbott 211.jpg|1. Normal heart. A, caval auricle; B, pulmonary veins auricle; C, right ventricle (caval blood ventricle) ; D, left ventricle (pulmonary blood ventricle).
File:Abbott 212.jpg|2. Embryonic heart. A, caval auricle; B, pulmonary veins auricle; C, right ventricle (caval blood ventricle) ; D, left ventricle (pulmonary blood ventricle).
File:Abbott 213.jpg|3. Mirror picture of normal heart. A, caval auricle; B, pulmonary veins auricle; C. right ventricle (caval blood ventricle) ; D, left ventricle (pulmonary blood ventricle).
File:Abbott 214.jpg|4. Pure congenital dextrocardia. A, caval auricle; B, pulmonary veins auricle; C, caval blood ventricle; D, pulmonary blood ventricle. Note similarity to No. 2.
File:Abbott 215.jpg|5. Graanboom's case of congenital dextrocardia (mirror picture). A, caval auricle; B, pulmonary blood auricle; C, caval blood ventricle; D, pulmonary blood ventricle.
File:Abbott 216.jpg|6. Grunmach's case of pure congenital dextrocardia (arrest in embryonic stage). E, vena cava superior; F, ventriculum dextrum; G, aorta; H , ventriculum sinistrum.
</gallery>


1, 2, 3, 4. Diagrams (1) showing normal heart; (2) embryonic heart; (3) mirror picture of normal
heart; (4) heart in usual type of pure congenital dextrocardia; (5) from Graanboom's case of pure
dextrocardia (mirror picture). Note that the apex is formed by the left (pulmonary blood) ventricle.
(From Nagel's article on Pure Congenital Dextrocardia in the Deutsches Archiv. f. klin. Med., xcvi, p.
572.) (6) Grunmach's case of pure congenital dextrocardia; arrest of development, persistent embrj-oni,
stage. Note that the apex is formed by the right (caval blood) ventricle. (From his article in the Ben.
klin. Wchnschr., 1890, page 22.) The auricles are designated "caval" and "pulmonary" (according to tae
veins they receive) , instead of right and left, in order to show the difference in situation in the mirror picture
and in Graanboom's case, from that of the usual type of pure congenital dextrocardia (Grunmach's jase).


Congenital dextrocardia, unassociated with other anomaly, is not itself of clinical significance and may be discovered accidentally with the heterotaxy that usually accompanies it, in a perfectly healthy subject, by physical and x-ray examination, which reveals the normal area of cardiac dulness on the right side, with corresponding location of sounds and fluoroscopic findings. Transposition of the heart, of the type usually associated with situs inversus viscerum (Graanboom's case) yields a characteristic electrocardiogram (Neuhof,^ Lewis^), in that Lead I, shows a reversal of all curves, and Leads II and III, replace each other. This supplies a diagnostic point between the transposed and non-transposed forms of Congenital Dextrocardia and also distinguishes the former from
a dextroversio, in which the electrocardiogram, although atypical, is not reversed. The curves in this latter condition have been studied by Neumann.^


[[File:Abbott_22.jpg|600px]]


'''Fig. 22'''
# Electrocardiogram from a case of congenital dextrocardia with transposed (mirror-picture) heart. Note that: Lead I, is completely reversed in that P, R, and T, normally directed upward in this lead, here point downward. Lead II, has changed places with Lead III.
# Electrocardiogram from a case of dextro-versio-cordis, i. e.. simple displacement of the heart to the right. Here there is no reversal of leads, but Lead I especially deviates from the normal in Q and R. (From Clinical Electrocardiography, by Thomas Lewis, London, 1913.)


Congenital dextrocardia, unassociated with other anomaly, is not itself of clinical significance and may be discovered accidentally with the
===Incomplete Heterotaxy===
heterotaxy that usually accompanies it, in a perfectly healthy subject,
by physical and .r-ray examination, which reveals the normal area of
cardiac dulness on the right side, with corresponding location of sounds
and fluoroscopic findings. Transposition of the heart, of the type usually
associated with situs inversus viscerum (Graanboom's case) yields a
characteristic electrocardiogram (Neuhof,^ Lewis^), in that Lead I, shows
a reversal of all curves, and Leads II and III, replace each other. This
supplies a diagnostic point between the transposed and non-transposed
forms of Congenital Dextrocardia and also distinguishes the former from
a dextroversio, in which the electrocardiogram, although atypical, is not
reversed. The curves in this latter condition have been studied by
Neumann.^


 
Of interest are three cases reported by Hickman, Royer and Wilson,<ref>Arch. Pediat., 1908, xxv, 882.</ref> and McCrae, of transposition of the viscera with only partial transposition of the heart, which maintained its normal position with apex pointing to the left, the ventricles and auriculoventricular ostia unchanged, but the auricles and great arterial trunks transposed, and the auricular septum defective. In McCrae's and Hickman's cases the pulmonary artery was atresic, in that by Royer and Wilson it was stenosed, and arose from the right ventricle with the aorta but in transposed relation. The ventricular septum was entire in McCrae's case, defective in the other two.
 
Fig. 22
 
 
 
1. Electrocardiogram from a case of congenital dextrocardia with transposed (mirror-picture) heart.
Note that: Lead /, is completely reversed in that P, R, and T, normally directed upward in this lead,
here point downward. Lead //, has changed places with Lead III.
 
2. Electrocardiogram from a case of dextro-versio-cordis, i. e.. simple displacement of the heart to
the right. Here there is no reversal of leads, but Lead I especially deviates from the normal in Q
and R. (From Clinical Electrocardiography, by Thomas Lewis, I-ondon, 1913.)
 
Incomplete Heterotaxy. — Of interest are three cases reported by  
Hickman, Royer and Wilson,^ and McCrae, of transposition of the  
viscera with only partial transposition of the heart, which maintained  
its normal position with apex pointing to the left, the ventricles and auriculoventricular ostia unchanged, but the auricles and great arterial trunks  
transposed, and the auricular septum defective. In McCrae's and Hickman's cases the pulmonary artery was atresic, in that by Royer and  
Wilson it was stenosed, and arose from the right ventricle with the  
aorta but in transposed relation. The ventricular septum was entire  
in McCrae's case, defective in the other two.


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^ Deutsch. nied. Wchnschr., 1912, xxxviii, p, 1920,  
^ Deutsch. nied. Wchnschr., 1912, xxxviii, p, 1920,  
^ Arch. Pediat., 1908, xxv, 882.






===Intrinsic Displacement of Chambers===


Intrinsic Displacement of Chambers. ^Four interesting examples, in anencephaly and ectopia cordis, of displacement of the chambers upon  
Four interesting examples, in anencephaly and ectopia cordis, of displacement of the chambers upon  
each other, as a result of extraneous mechanical force exerted on the heart after it was fully formed, are reported by Jane Robertson.^  
each other, as a result of extraneous mechanical force exerted on the heart after it was fully formed, are reported by Jane Robertson.^


==Anomalies of the Heart as a Whole==
==Anomalies of the Heart as a Whole==
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===Diverticulum===
===Diverticulum===


The heart may be prolonged into a hollow process.  
The heart may be prolonged into a hollow process. Arnold"- reports a female child, aged two and a half months, a subject of congenital lues, in whom the apex of the left ventricle ran out into a hollow process which bent around hke a hook, its blind end projecting upward and to the left. In Koller-Aeby's^ case it formed a pulsating process with muscular walls descending from the apex of the left ventricle through a defect in the diaphragm to form part of an umbilical hernia.  
Arnold"- reports a female child, aged two and a half months, a subject  
of congenital lues, in whom the apex of the left ventricle ran out into a  
hollow process which bent around hke a hook, its blind end projecting  
upward and to the left. In Koller-Aeby's^ case it formed a pulsating  
process with muscular walls descending from the apex of the left ventricle  
through a defect in the diaphragm to form part of an umbilical hernia.  
Diverticulum of the heart is rare and of no clinical significance.  
Diverticulum of the heart is rare and of no clinical significance.  


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diffuse myomatous infiltration. Other typical cases of congenital  
diffuse myomatous infiltration. Other typical cases of congenital  
hypertrophy without valvular disease, nephritis, or apparent cause, are  
hypertrophy without valvular disease, nephritis, or apparent cause, are  
reported by Effron,'* Kalb,"^ and Ratner.^
reported by Effron,'* Kalb,<ref>Munich Thesis, 1906.</ref> and Ratner. <ref>Berlin Thesis, 1912.</ref>




===Congenital Rhabdomyoma===
===Congenital Rhabdomyoma===


An interesting problem is presented by  
An interesting problem is presented by the occurrence in infants or young children of embryonic muscle tumors of the heart wall associated, in the majority of the cases, with sclerosis of the cerebral cortex. The condition was first described by von Recklinghausen and by Virchow and was first reported on this Continent by Knox and Schorer.<ref>Arch. Pediat., 1906. xxiii, 361.</ref> Wohlbach<ref>Jour. Med. Research., 1907, xvi, 495. </ref> has recently studied the 11 cases on record, and has added an observation of his own of an instance in which  
the occurrence in infants or young children of embryonic muscle tumors  
of the heart wall associated, in the majority of the cases, with sclerosis  
of the cerebral cortex. The condition was first described by von Recklinghausen and by Virchow and was first reported on this Continent by  
Knox and Schorer.^ Wohlbach^ has recently studied the 11 cases on  
record, and has added an observation of his own of an instance in which  
a rhabdomyoma of the wall of the right ventricle was associated with  
a rhabdomyoma of the wall of the right ventricle was associated with  
multiple nests of neuroglia in the spinal meninges. The child, a  
multiple nests of neuroglia in the spinal meninges. The child, a  
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1 Virchows Arch., 1894, cxxxvii, 318. ^ Arch. f. Gyndk., 1907, Ixxxii, 185.  
1 Virchows Arch., 1894, cxxxvii, 318.  
^ Arch. f. Gyndk., 1907, Ixxxii, 185.  


^ Munchen. med. Wchnschr., 1898. ^ Zurich Thesis, 1903.  
^ Munchen. med. Wchnschr., 1898.  
^ Zurich Thesis, 1903.


s Munich Thesis, 1906. ^ Berlin Thesis, 1912.


7 Arch. Pediat., 1906. xxiii, 361. ^ Jour. Med. Research., 1907, xvi, 495.


An explanation of this remarkable combination of congenital cerebral sclerosis with rhabdomyoma may be sought in the fetal malnutrition invariably present, which may have led to the vascular degeneration that must underlie such changes (Wohlbach).


An explanation of this remarkable combination of congenital cerebral
 
sclerosis with rhabdomyoma may be sought in the fetal malnutrition
----
invariably present, which may have led to the vascular degeneration
that must underlie such changes (Wohlbach).




<references/>
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Abbott ME. Congenital Cardiac Disease (1915) Osler & Mccrae's Modern Medicine 6, 2nd Edition.

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1915 Congenital Cardiac: Congenital Cardiac Disease | Heart Development | Literature | Etiology | Cyanosis | Classification | Pericardium | Heart Displacement | Whole Heart | Anomalous Septa | Interauricular Septum | Interventricular Septum | Absence of Cardiac Septa | Aortic Septum | Pulmonary Stenosis and Atresia | Pulmonary Artery Dilatation | Aortic Stenosis or Atresia | Primary Patency and Ductus Arteriosus | Aorta Coarctation | Aorta Hypoplasia | Diagnosis Prognosis and Treatment | Figures | Embryology History | Historic Disclaimer

Classification

Many attempts have been made to reduce cardiac anomalies to a scientific classification, but none have been entirely successful. The most logical arrangement is undoubtedly one based upon the stage of development at which the defect has occurred, differentiating those cases due to arrest of growth from those originating at a period of intrauterine life and apparently caused by fetal disease. Our knowledge of the development of the heart is still too limited to permit of a complete classification on this basis, and, on the other hand, the etiology of a given condition is often impossible to decide in the individual case. Moreover, a grouping based on development alone is sometimes unpractical, for widely different pathological results may ensue from arrest of different parts of the heart at about the same period of fetal life. For these reasons no one classification will be found adequate in its practical application, and it would almost seem that a grouping "on mixed principles" is the only one under which all the cases can be satisfactorily placed.


The 631 cardiac defects here studied present, either as the primary lesion or as a complicating condition, illustrations of practically all the cardiac anomalies known. In the tables^ of relative frequency, age, sex, etc., pages 342 and 343, an attempt is made to arrange these cases in as logical an order as possible. It represents an attempt at a detailed classification based on strictly anatomical principles and on development so far as this is known, but without regard to the etiology, which is so often obscure. Thus the group of stenosis and atresias of valvular orifices is subdivided, not into inflammatory and developmental forms and their subdivisions, with or without transposition or rechtslage (Rauchfuss, Vierordt), but on anatomical considerations only, with regard to the seat of the stenosis and the presence or absence of septal defects.

Abbott table1.jpg


1 Note. - "Double" indicates a systolic with a diastolic murmur. The sex is not always mentioned in the records of cases. The nmnbers in these columns do not, therefore, always correspond to the totals in the chart. The data given as to age, sex, postmortem findings, and clinical aspects refer to the cases in the first column in the chart. This column is repeated in the third to last column, and this, added to the second to last column, gives the total relative frequency of each defect, whether classified as primary lesion or as complicating other defects.


The majority of cardiac defects are complicated, and it is often difficult, sometimes impossible, to say which is the primary lesion; many of the eases present other anomalies of equal importance with those with which they are grouped. A cross-index has therefore been made in the two columns next to the end, and the total relative frequency is to be found in the last column of the chart.

Anomalies of the Pericardium

Absence or Defect

The Pericardium may be entirely absent, as in some forms of ectopia cordis, or its parietal layer may be more or less defective. Some forty-two cases are on record and the subject has recently been reviewed by Ebstein,^ Plaut,^ McGarry,^ and Cameron. The defect always involves the left side of the sac but it may vary from a localized hole with smooth edges lying over the pulmonary artery opposite the root of the left lung and communicating with the left pleura, to complete absence of the parietal layer, the heart lying in the anterior mediastinum, without any serous envelope. In all the cases recorded except the four above mentioned, the whole left and anterior walls of the pericardium are wanting, and the heart and left lung lie in a common cavity, the left pleura being continuous with the epicardium and the anterior and right walls of the sac being represented by one or more rudimentary folds; in the best developed cases two folds, one sagittal and one crescentic; spring from the diaphragm and are inserted, the one into the anterior mediastinum and the other posteriorly at or near the root of the left lung; in the more extreme cases the diaphragm is free and a serous fold encircles the base of the heart with the great trunks; in complete defects no trace of the parietal layer may exist, or it may be represented by a few fatty appendices arranged fringe-like about the base of the heart. The left phrenic nerve maintains its relationship to the pleuropericardial septum, running down parallel to the border of the sagittal rudimentary fold when this is present, and is displaced to the right in a degree increasing with the degree of the defect. This anomaly in the course of the nerve, and the serous investment of the margins of the defect, are points diagnostic of its congenital origin.


Pathogenesis

An explanation of the condition is to be sought toward the end of the fifth week of fetal life, when the pericardial begins to be separated from the pleural coelom. At this time the so-called pulmonary ridge, an elevation from the walls of the ducts of Cuvier, encircles, and gradually obliterates, the canal of communication between the two cavities, and descends to the septum transversum or primitive diaphragm as the pleuropericardial septum. Under normal conditions the left duct of Cuvier atrophies in early fetal life. Pericardial defect, with its invariable relation to the left pleural cavity, may well be due, as suggested by Perna, to a too early atrophy of this structure, dependent upon some anomaly of circulation in the great venous trunks, the arrested development of the pulmonary ridge resulting leading to a localized defect (pleuropericardial foramen), or to the more or less complete absence of the pleuropericardial septum. In one of Keith's cases, a defect of the left pleuroperitoneal membrane is also to be concluded, for the heart, left lung, liver, spleen, and stomach lay in the left pleuropericardial cavity.


1 Munchen. med. Wchnschr., 1910, Ivii, 522.

2 Frankf. Zeitsch.f. Path., 1913, xii, 141.

3 Anat. Record, 1914, February 20.

^ Trans. Chicago Path. Soc, 1914, ix, p. 148.


Clinical Aspects

The defect in itself has no direct effect upon the heart, the patients reaching adult life, and sometimes even attaining advanced age without any untoward sign. Its clinical significance depends chiefly upon two factors: First of these is the abnormal juxtaposition of the heart and left lung, whereby the heart is exposed, without the protection of its serous envelope, to the many inflammatory changes prone to attack the pleura; some cause also, whether traction from adhesions with the constantly pulsating heart or friction with the surface of this, appears to act deleteriously upon the left lung and pleura, predisposing these to disease. Among the forty cases recorded the incidence of left-sided pulmonary complications is extremely large. Picchi's two patients died of left lobar pneumonia, Weisbach's of left purulent pleurisy, Powell's of left pneumothorax. Plant's second case and Faber's of acute left-sided pleurisy and Baly's, Hughes', and Saxer's of pulmonary tuberculosis.


Secondly, the cor mobile resulting frorn the lack of pericardial attachment to the diaphragm and mediastinal structures, may lead to sudden kinking of the great vessels resulting not only in s3^mptoms of precordial distress (Ebstein), but apparently, in one instance at least in death itself. In the case recorded by Boxall death occurred on the third day after confinement, apparently from the sudden slipping of the heart's apex out of a low-walled pouch formed by two rudimentary folds attached to the diaphragm, in the altered intrathoracic pressure following delivery. Thirty hours before death urgent dyspnoea, collapse, and a systolic murmur over the precordium suddenly appeared. Pulmonary thrombosis was suspected, but was not confirmed at autopsy, when the heart was found in the left pleura. The number of fatalities is high.


Diagnosis

This is difficult, but does not seem impossible. The greatly increased mobility of the heart, its occasional hi^'pertrophy either from this cause or from the traction of inflammatory^ fibrous tissue bands attaching it to the left pleura and diaphragm, and its frequent displacement to the left through such traction or from similar causes are the chief points. In spite of its rarity, pericardial defect should always be considered in the light of the .T-ray findings.

In Faber's case, a man of fifty-one years, in good health until five weeks before death, when an extensive left pleural eft'usion developed, the heart was only moderately displaced to the right before aspiration, although the left chest was entirely dull; after the withdrawal of 2200 cc. of fluid the apex was evident in the third and fourth interspaces in the left anterior axillary line, and at a later aspiration of 3100 cc, the heart was felt in this region by the aspirating trocar. These facts supplied a basis for a correct diagnosis.


Unattached Pericardium

Turner describes an adult male subject in whom the parietal pericardium was perfectly free from continuity with the central tendon of the diaphragm or other structure, and was attached only about the base of the great vessels, closely embracing the heart, which could be drawn completely out of the chest (cor viohile), . the inferior vena cava lay free in the thorax for an inch before piercing the pericardium. A similar condition was observed in the w^alrus and was thought here to be normal.

Diverticuluin or Hernia Pericardii. - This consists in a localized bulging of the serous, or serous and fibrous, coats of the parietal layer, whereby a thin-walled cyst is formed, communicating with the pericardial cavity either directly by a narrow orifice, or by a tubular canal. An interesting example was seen by the writer in the Museum at Bologna, from a case published by Coen.^ In this specimen a unilocular chamber of a capacity of 40 cc, formed by the protrusion of the serosa, projected from the right border of the parietal pericardium, as a kidney shaped tumor 17.5 cm. in circumference, and communicated with the interior of the pericardial sac by a minute orifice lying at the bottom of a small fossa. Coen gives an analysis of ten other cases in the literature.

This condition is of little clinical importance, usually remaining latent. Secondary pathological changes, such as calcification or fibrosis of the cyst walls, or occlusion of its orifice and consequent distension of its cavity may supervene, and make it an impediment to the heart's action. The possibility of a pericardial diverticulum should not be overlooked in the diagnosis of mediastinal tumors.


Displacements of the Heart

Ectopia Cordis

By this term is understood a displacement so that the heart passes out of the thorax, and comes to lie either upon the outer surface of the body or in the abdominal cavity. Full bibliographical studies, with reports of cases, are given by Jones,^ and by Ellis.^ The latter, following Rauchfuss, recognizes three forms, (1) cervical heart, in which the organ lies high up in the neck; (2) pectoral heart with fissure of the sternmn, and (3) abdominal heart in which the heart is projected through a defect in the diaphragm into the abdominal cavity.

The existence of cervical heart, or of pectoral heart with fissure of the upper part of the sternum and absence of pericardium, is not compatible with life; in abdominal heart on the other hand life is not necessarily shortened (witness Deschamps' case of a healthy soldier whose heart occupied the position of the left kidney in the lumbar region), and in pectoral heart with inferior sternal fissure and pericardium present, it might with due care be indefinitely prolonged. In Goode's^ patient, with fissure of the sternum below the manubrium, the heart was oiled every three hours and kept covered by a cardboard box, and the child did very well for a time. Ellis quotes a case in which a thin membrane covering the heart sloughed on the fifteenth day, and the outer skin covering the organ was successfully united by sutures. He suggests that such operative interference might be tried in suitable cases where the visceral pericardium is present.



^ Bull, della Soc. Med. Chi. di Bologna, 1885, xv, Fasc. 1.

2 Trans. Path. Soc, London, 1869, xx.

3 Proc. Path. Soc, Philadelphia, 1906, p. 36.

  • Virginia Med. Semi-Monthly, 1904, p. 555.


Dextrocardia

This term is applied clinically to all cases in which the heart is found displaced to the right side of the thorax. Pathologically two conditions must be distinguished . In the one the heart is simply displaced to the right as a result, usually, of acquired disease {De.rtroversio cordis). In the other the organ is also more or less altered in its position in the thorax, so that the apex comes to point to the right. In these latter cases two forms must again be recognized :

A. The heart is not transposed, but appears to have undergone a simple rotation from left to right on its vertical axis, so that its left chambers come' to lie more' anteriorly and its right chambers more posteriorly. The apex points to the right but is formed not of the left but of the right (venous) ventricle, which remains on the right side and receives blood from the right (venous) auricle into which the venae cavae empty. This, which is not a true transposition, is the condition present in the majority of the cases of congenital dextrocardia. It is well illustrated by Grunmach's^ case (See Fig. 21.)
B. The heart may he completely reversed upon itself, those parts normally upon the left coming to lie on the right side, but the relation of the various structures to each other remaining unchanged, so that a complete mirror picture of the. normal, heart results. Here the apex, pointing to the 'bright, is formed of what was normally the left ventricle, which now lies on the right side and communicates with the right (normally left) auricle which receives the pulmonary veins and is structurally the systemic auricle. In complete situs inversus this "mirror" condition is the rule, but true transposition of the heart only, without situs inversus is exceedingly rare, the only cases we know of on record being these of Graanboom^ and Rokitansky.


The etiology of these two t^^pes of congenital dextrocardia differs widely. Nagel reviews six cases with clinical and autopsy reports in the literature, and demonstrates, with the aid of diagrams, that five of these, namely, those by Grunmach, Geipel, Lowenthal, Barmwerth and himself, belong to the type A above, in which the heart is not transposed, but presents simply a persistence of the embryonic stage, in which the apex was formed by the right half of the common ventricle. The fact that in all five of these cases the grave anomalies of pulmonary stenosis and ventricular septal defect were associated, affords convincing proof that a primary arrest of development had occurred.


In the sixth case, on the other hand, that by Graanboom, in which there was true transposition of the heart, and the septiun was entire, the probable explanation is to be sought, not in an arrest of development, but, with the causes of transposition in general, in the altered relation of the embryo to the primitive chorionic villi.


^ Berlin, klin. Wchnschr., 1890, No. 2.

2 Zeitschr. f. klin. Med., 1891, Bd. 18, 2.

^ Deut. Arch. f. klin. Med., vol. xcvi, p. 552.


Abbott 21.jpg

Fig. 21 1, 2, 3, 4. Diagrams (1) showing normal heart; (2) embryonic heart; (3) mirror picture of normal heart; (4) heart in usual type of pure congenital dextrocardia; (5) from Graanboom's case of pure dextrocardia (mirror picture). Note that the apex is formed by the left (pulmonary blood) ventricle. (From Nagel's article on Pure Congenital Dextrocardia in the Deutsches Archiv. f. klin. Med., xcvi, p. 572.) (6) Grunmach's case of pure congenital dextrocardia; arrest of development, persistent embryonic stage. Note that the apex is formed by the right (caval blood) ventricle. (From his article in the Ben. klin. Wchnschr., 1890, page 22.) The auricles are designated "caval" and "pulmonary" (according to the veins they receive) , instead of right and left, in order to show the difference in situation in the mirror picture and in Graanboom's case, from that of the usual type of pure congenital dextrocardia (Grunmach's jase).



Congenital dextrocardia, unassociated with other anomaly, is not itself of clinical significance and may be discovered accidentally with the heterotaxy that usually accompanies it, in a perfectly healthy subject, by physical and x-ray examination, which reveals the normal area of cardiac dulness on the right side, with corresponding location of sounds and fluoroscopic findings. Transposition of the heart, of the type usually associated with situs inversus viscerum (Graanboom's case) yields a characteristic electrocardiogram (Neuhof,^ Lewis^), in that Lead I, shows a reversal of all curves, and Leads II and III, replace each other. This supplies a diagnostic point between the transposed and non-transposed forms of Congenital Dextrocardia and also distinguishes the former from a dextroversio, in which the electrocardiogram, although atypical, is not reversed. The curves in this latter condition have been studied by Neumann.^

Abbott 22.jpg

Fig. 22

  1. Electrocardiogram from a case of congenital dextrocardia with transposed (mirror-picture) heart. Note that: Lead I, is completely reversed in that P, R, and T, normally directed upward in this lead, here point downward. Lead II, has changed places with Lead III.
  2. Electrocardiogram from a case of dextro-versio-cordis, i. e.. simple displacement of the heart to the right. Here there is no reversal of leads, but Lead I especially deviates from the normal in Q and R. (From Clinical Electrocardiography, by Thomas Lewis, London, 1913.)

Incomplete Heterotaxy

Of interest are three cases reported by Hickman, Royer and Wilson,[1] and McCrae, of transposition of the viscera with only partial transposition of the heart, which maintained its normal position with apex pointing to the left, the ventricles and auriculoventricular ostia unchanged, but the auricles and great arterial trunks transposed, and the auricular septum defective. In McCrae's and Hickman's cases the pulmonary artery was atresic, in that by Royer and Wilson it was stenosed, and arose from the right ventricle with the aorta but in transposed relation. The ventricular septum was entire in McCrae's case, defective in the other two.


1 Jour. Amer. Med. Assn., 1913, Ix, p. 1064.

2 Clinical Electrocardiography, 191.3, p. 100.

^ Deutsch. nied. Wchnschr., 1912, xxxviii, p, 1920,


Intrinsic Displacement of Chambers

Four interesting examples, in anencephaly and ectopia cordis, of displacement of the chambers upon each other, as a result of extraneous mechanical force exerted on the heart after it was fully formed, are reported by Jane Robertson.^

Anomalies of the Heart as a Whole

Acardia, Hemicardia

Where grave interference with the circulation occurs at a very early embryonic period, the heart may not develop at all (acardia), or it may be rudimentary (hemicardia). Such a condition usually develops in one member of a uniovular twin pregnancy in which anastomosis of the vessels of .the two individuals can take place through their common placenta.


All degrees of acardia may occur and from the developmental standpoint the findings are extremely interesting. Thus Campbell and Shepherd report a case in which the circulation had been interfered with before the heart had formed at all, and in which the two dorsal arteries passing from the umbilical opening toward the head of the amorphous monster, represented a persistence of the early vitelline circulation. Kehrer^ adds to an analysis of thirteen cases from the literature an account of a case of his own which is remarkable for the advanced state of the rudimentary organ. Cases not included in Kehrer's review are reported by Schubert^ and by Nacke and Benda.


Multiple Hearts

This phenomenon is commoner in animals. An explanation is perhaps to be sought in an irregular division of the omphalomesenteric or vitelline veins, from which the first heart milage is derived.


Bifid Apex

This may occur without other anomaly or complicating grave defects. The apex of the early embryonic heart during its amphibian stage, is bifid; a condition to be ascribed to the rapid downward growth of the apices of both ventricles at this time, so that a deep interventricular groove or cleft is formed. Obliteration of this cleft takes place in embryos over 11 mm. long, by the development downward of the embryonic apex, which occurs simultaneously with the closure of the interventricular foramen. In the adult mammalian heart bifid apex is thus to be ascribed to a persistence of the interventricular groove (Gegenbaur), due to an arrest of development of the embryonic apex, as is evidenced by the absence of the muscular vortex normally present here, each apex in the bifid state being formed independently from musculature derived respectively from the musculospiral and sinospiral bands (Mall) .^ A deeply bifid apex is normal in the dugong; it occurs in 3 of Theremin's 106 cases and in 15 of our series.


1 Jour. Path, and Bad., 1913, xviii, 211. ^ ^^^h. J. Gynak., 1908, Ixxxv, 121.

3 Monatsch. f. Geburts., 1908, xxviii, 3. ^ Zentralbl. f. Gynak., 1907, p. 17.

5 Anat. Rec, 1912, vi, 167.


Diverticulum

The heart may be prolonged into a hollow process. Arnold"- reports a female child, aged two and a half months, a subject of congenital lues, in whom the apex of the left ventricle ran out into a hollow process which bent around hke a hook, its blind end projecting upward and to the left. In Koller-Aeby's^ case it formed a pulsating process with muscular walls descending from the apex of the left ventricle through a defect in the diaphragm to form part of an umbilical hernia. Diverticulum of the heart is rare and of no clinical significance.


Primary Congenital Hypertrophy

Recently a number of observations have been published in which hypertrophy of the heart existed at birth. In 1898, Simmonds^ reported the first clear case of primary congenital hypertrophy. In a newly born child which died during a protracted labor all the organs including the kidney were normal, but the heart was greatly enlarged. It weighed 44 gm. (normal weight 19 to 20 gm.); the right ventricle was 0.75 to 1 cm., the left 1 to 1.25 cm. thick. The papillary muscules were small and took no part in the hypertrophy, thus indicating that this was not caused by overwork. Simmonds suggests that the hypertrophy may have been due either to some cause in early embryonic life, which may later pass away, or to Mrchow's diffuse myomatous infiltration. Other typical cases of congenital hypertrophy without valvular disease, nephritis, or apparent cause, are reported by Effron,'* Kalb,[2] and Ratner. [3]


Congenital Rhabdomyoma

An interesting problem is presented by the occurrence in infants or young children of embryonic muscle tumors of the heart wall associated, in the majority of the cases, with sclerosis of the cerebral cortex. The condition was first described by von Recklinghausen and by Virchow and was first reported on this Continent by Knox and Schorer.[4] Wohlbach[5] has recently studied the 11 cases on record, and has added an observation of his own of an instance in which a rhabdomyoma of the wall of the right ventricle was associated with multiple nests of neuroglia in the spinal meninges. The child, a female, with hydrocephalus and spina bifida and paralysis of the lower half of the body from birth, died of scorbutus at ten months. The heart was very large, weighing 72.5 gm., with hypertrophied papillary muscles. Just below the pulmonary valve an ovoid nodule, 1.7 x 4 cm. wide, of grayish-red color and elastic consistence, lay imbedded in the interventricular septum and in the papillary muscle of the anterior tricuspid valve segment, to which it supplied chordse. Microscopically it was composed of a delicate reticulum of connective tissue supporting heterogeneous cells having a more or less irregularly striated fibrillary matrix and containing in many instances large intracellular spaces and peripherally placed nuclei, the whole bearing a bizarre resemblance to the modified muscle cells (Purkinje fibres) of the adult heart.



1 Virchows Arch., 1894, cxxxvii, 318. ^ Arch. f. Gyndk., 1907, Ixxxii, 185.

^ Munchen. med. Wchnschr., 1898. ^ Zurich Thesis, 1903.


An explanation of this remarkable combination of congenital cerebral sclerosis with rhabdomyoma may be sought in the fetal malnutrition invariably present, which may have led to the vascular degeneration that must underlie such changes (Wohlbach).




  1. Arch. Pediat., 1908, xxv, 882.
  2. Munich Thesis, 1906.
  3. Berlin Thesis, 1912.
  4. Arch. Pediat., 1906. xxiii, 361.
  5. Jour. Med. Research., 1907, xvi, 495.

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Abbott ME. Congenital Cardiac Disease (1915) Osler & Mccrae's Modern Medicine 6, 2nd Edition.

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