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An unusual case of an interatrial communication similar to a sinus venosus defect associated with an overriding pulmonary venous chamber is reported. There was no direct connection of the pulmonary venous system with either the superior vena cava or the low right atrium, as is usual with sinus venosus defects. This defect may represent the result of a sinus venosus defect associated with cor triatriatum.
Hemoptysis is most commonly due to pulmonary pathology. When cardiac in origin it is usually due to mitral valve disease. The authors present two unusual causes of hemoptysis: cor triatriatum and primary cardiac liposarcoma.
We describe an infant with hypoplasia of the left heart diagnosed prenatally who, at birth, had signs of severe pulmonary venous obstruction. Echocardiography indicated normally connecting pulmonary veins, and showed a paradoxical right-to-left shunt across a patent oval foramen. Postmortem examination revealed that the obstruction was due to a divided left atrium, or cor triatriatum sinister, with an imperforate muscular diaphragm separating completely the two components of the divided atrium.
In 18 of 77 adult patients presenting with congenital heart disease, three-dimensional conceptualization was found to be insufficient using two-dimensional echocardiographic imaging. In these individuals, three-dimensional image reconstruction from digitized parallel images was carried out, providing additional information in 14 of them. Three-dimensional echocardiography and volumetry may, especially in patients with atrial septal defect, cor triatriatum and corrected transposition of the great arteries, be an asset amplifying the diagnostic information yielded by conventional two-dimensional echocardiographic approaches.
Congenital anomalies of the systemic venous connection to the heart represent a rather wide and heterogeneous group of malformations, whose physiological consequences may vary from nil to the most severe form of systemic arterial desaturation. The malformations may be summarized as follows: (1) Left superior vena cava connected to the coronary sinus, interrupted inferior vena cava and absent right superior vena cava that do not indicate surgical repair 'per se', but require some technical attention during open heart surgery performed for other anomalies; (2) Left superior vena cava connected to the left atrium, due to incorporation of the coronary sinus into the left atrial cavity, resulting in a right-to-left-shunt; (3) Right superior vena cava or inferior vena cava draining into the left atrium, both are extremely rare and require treatment for the ensuing right-to-left shunt; (4) Total anomalous systemic venous connection to the left atrium, usually combined with atrial isomerism and other very complex heart malformations; (5) Cor triatriatum dexter, which has been frequently diagnosed as an anomalous venous connection for its similar hemodynamic consequences. Such anomalies are reviewed with particular respect to their surgical implications.
A case of right lung hypoplasia and dyspnea, presented at our institution for cardiac evaluation, is described. Clinical evaluation and instrumental findings of normally connected but completely obstructed right pulmonary venous drainage have led us to diagnose an exceptional variant of subtotal cor triatriatum. Differential diagnosis was mainly carried out between pulmonary venous anomalies and drainage obstructions. Symptom relief was obtained with surgical excision of the atrial dividing membrane.
In this report we describe the two-dimensional echocardiographic features of a case of cor triatriatum dexter. In this situation the right atrium is divided by a membrane in two chambers. Considering the anatomic characteristics of this case we added a new type in the classical classification proposed by Doucette et al.
An acquired form of cor triatriatum, resulting in significant supramitral valve obstruction, complicating a pediatric orthotopic heart transplantation was diagnosed by transesophageal echocardiography. Surgical resection of the hypertrophied atrial septum and atrial wall corrected the anatomic obstruction and improved the hemodynamics.
A case of persistent right valve of the sinus venosus (the so-called cor triatriatum dexter) is described. There were associated severe pulmonary valve stenosis and probe patent foramen ovale. The patient survived to the age of 25, without complaints. His death was due to complications of pulmonary thromboembolism. The anomaly was detected prior to death by echo-doppler-cardiogram. The postmortem findings of the heart are described in detail.
The unique anatomy, physiology, and surgical repair of a patient with features of three rare congenital heart defects (total anomalous pulmonary venous return to the coronary sinus, cor triatriatum, and unroofed coronary sinus with persistent left superior vena cava to the left atrium) is described. Analysis of this case suggests that these three conditions are linked in that they all may result as a consequence of a defect in the same embryologic process (i.e., incorporation of the pulmonary venous confluence into the left atrium).
We report 4 cases of congenital and acquired coronary sinus ostial atresia incidentally found during electrophysiologic assessment for supraventricular arrhythmias. Congenital variants consisted of coronary sinus drainage predominantly via persistent left superior vena cavae and partial coronary sinus unroofing into the left atrium. The acquired variant was inadvertently produced during surgery for cor triatriatum. A variety of electrophysiologic substrates including right and left-sided accessory pathways and both typical and atypical AV nodal reciprocating tachycardia were identified. Approaches to imaging and accessing the coronary sinus when the os cannot be cannulated are discussed, including a search for right atrial accessory venous collaterals, venography to rule-out a persistent left superior vena cava, and coronary angiography.
Between 1978 and 1987, 39 patients aged 1 day to 15 years underwent surgery for symptomatic left ventricular inflow obstruction. Four diagnostic groups were identified: cor triatriatum (6 patients), supravalvar mitral membrane (SVMM) with a normal mitral valve (7 patients), SVMM with an abnormal mitral valve (9 patients) and mitral stenosis (17 patients). Associated cardiac anomalies occurred in 26 patients (67%). There were 8 deaths (21%), 3 in patients with SVMM and an abnormal mitral valve and 5 in patients with mitral stenosis. Survival for patients with normal mitral valves was significantly better than that for patients with abnormal mitral valves (13/13 vs 18/26, P less than 0.05). There was also high morbidity in patients requiring prosthetic mitral valve replacement. These data suggest that the outcome of surgical treatment for left ventricular inflow obstruction may be predicted according to the site of the obstruction. This is best determined preoperatively by cross-sectional echocardiography which allows optimal planning of surgical strategy.
BACKGROUND: Pulmonary venous obstruction (PVO) induces pulmonary arterial hypertension, as well as pulmonary venous hypertension, and jeopardizes the repair of cardiac lesions. METHODS: Four cases of congenital mitral stenosis and 4 cases of cor triatriatum (Lucas type A), ages ranging from 2 months to 16 years, were histologically examined on pulmonary vasculature. Histometrical analysis was performed on medial thickness and intimal changes of both pulmonary arteries and veins. For comparison, the examination of pulmonary vasculature in ventricular septal defect (VSD) cases was also performed. RESULTS: Medial thickening and intimal fibrosis, in both pulmonary arteries and veins with widespread lymphangiectasia, were characteristic vascular changes of PVO cases. Medial thickness of pulmonary arteries was correlated with preoperative pulmonary arterial pressure (PAP) (r = 0.77, p = 0.03 for systolic PAP), and greater than that of VSD cases. Medial thickness of pulmonary veins was also greater in PVO cases. Intimal fibrosis of pulmonary arteries and veins was seen extensively at the advanced ages, whereas no plexiform lesions or more advanced stages of pulmonary vascular disease were present. CONCLUSIONS: Congenital PVO induced progressive medial thickening and intimal fibrosis in pulmonary arteries and veins accompanied by lymphangiectasia. However, no plexiform lesions or more advanced stages of pulmonary vascular disease were present, which may explain the reversibility of pulmonary hypertension due to congenital PVO.
Six hearts specimens of cor triatriatum dextrum, eight with the Chiari's network, and 3 with a membranous remnant of the crista terminalis are studied. These anomalies are interpreted as varying degrees of persistence of the right valve of the sinus venous which reflect different stages of their morphogenesis. The most frequent congenital heart defects associated with these sinus remnants are the absence of a right atrioventricular connexion and pulmonary atresia with intact ventricular septum. There may be some haemodynamic factors in the right atrium such as partial of complete obstruction between the atrium and the right ventricle which explain the fact that the valve is not reabsorbed. In our specimens the persistent right valve divided the right atrium into two compartments; one is located in the medial sinusal portion where both cavae veins and coronary sinus are connected; the other is lateral compartment which represents the primitive right atrium. It must be noted that the persistent right valve favors blood flow to the left atrium through the patent foramen ovale or through an atrial septal defect deviating the course of the blood away from the right ventricle. This could cause the hypoplasia of the right ventricle. When the valve bulges it obstructs the blood flowing into the right ventricle. The diagnosis of these malformations can be made by echocardiographic, angiocardiographic procedures and nuclear magnetic resonance.
The clinical presentation of right ventricular (RV) dysfunction due to congenital heart disease (CHD) is similar to that of cor pulmonale. RV volume and pressure loads, and primary RV myocardial dysfunction are mechanisms by which CHD affects right heart function. RV volume load may arise from pre-tricuspid left to right shunts (e.g., atrial septal defect) or regurgitant lesions in the right heart (e.g., Ebstein's anomaly of the tricuspid valve and pulmonary insufficiency after repair of tetralogy of Fallot). RV pressure load may be caused by anatomic obstruction to RV outflow and by pulmonary arteriolar hypertension. The latter can result from Eisenmenger syndrome secondary to congenital and postoperative left to right shunts or from defects that cause pulmonary venous hypertension (e.g., pulmonary vein stenosis, cor triatriatum, or mitral stenosis). The RV myocardium may be affected by a primary cardiomyopathy or by congenital abnormalities of the coronary vessels. Finally, CHD may be associated with airway obstruction, scoliosis, or pulmonary emboli, which, in turn, may lead to the development of cor pulmonale. Congenital heart disease, therefore, must be included in the differential diagnosis of patients who present with right ventricular dysfunction.
From 1975 to 1980, 106 infants in the first year of life with complex cardiac anomalies underwent isolated intra-atrial repair; 70 were in the first 3 months of life. Of these 70, 39 were 1 to 3 months old, 15 were 1 week to 1 month old, and 16 were less than 1 week old. The median age was 70 days, the range, 1 to 360 days. These infants included 74 with transposition of the great arteries, 28 with total anomalous pulmonary venous connection, and four with complex lesions (cor triatriatum and combined total anomalous pulmonary venous connection and anomalous systemic connection). Overall survival was 95%; for those in the first 3 months of life, 96%; for those in the first month, 94%; and for those in the first week, 100%. Age was not a determinant of survival. Attempts to delay surgical intervention once symptoms appear only results in clinical deterioration and interim mortality.
Cranial neural crest, which extends from the mid-diencephalon to somite five, plays an integral role in development of pharyngeal arch derivatives and supplies mesenchyme to the aortic arch arteries. Neural crest cells in pharyngeal arches three, four, and six migrate to the heart and are involved in aorticopulmonary and conotruncal septation. Ablation of the "cardiac" neural crest cells in chick embryos results in a variety of outflow tract anomalies, including persistent truncus arteriosus. Although other studies have shown the importance of the neural crest in the development of the cardiac outflow tract, the role of neural crest in venous development has not been established. This investigation evaluates the effect of cardiac neural crest ablation on the morphological development of the pulmonary vein. The presence of the pulmonary vein was confirmed initially at early stage 15 using histological sections and computer reconstructions of serially sectioned, normal embryos. India ink injections demonstrated a complete, patent pulmonary circuit at stage 18. Cardiac neural crest was ablated at stages 8-10. Operated, sham-operated, and control embryos were sacrificed at incubation day 11, and acrylic plastic casts prepared of the intravascular compartment. In experimental embryos with persistent truncus arteriosus, there were no morphological differences in the pulmonary veins, compared with shams and controls. These data indicate that the lesions of the cardiac neural crest have little morphological impact on pulmonary vein development. It is concluded that alterations in the cardiac neural crest are not involved in venous anomalies such as cor triatriatum and total or partial anomalous pulmonary venous return.
The fibrous skeleton of the human heart is composed of several parts which are formed from different, mainly extracardiac sources. The atrioventricular valve rings are formed by invagination of sulcus tissue at the atrioventricular and bulboventricular transitions. The atrial part of the central fibrous body is formed from an ingrowth of tissue from the dorsal mesocardium, the ventricular part from ingrowth of sulcus tissue towards an endocardial structure, the left bulbar ridge. During valve development the atrioventricular endocardial cushions, initially situated between the developing atrial and ventricular parts of the central fibrous body, are almost completely removed downwards into the ventricular cavities. However, a small portion of these cushions remains on top of the ventricular septum, is "trapped" by the surrounding sulcus tissues and becomes incorporated in the central fibrous body. These embryological findings on this centrally located area of the heart have implications for certain types of cardiac abnormalities. In this respect the atrioventricular conduction system, cor triatriatum sinistrum and atrioventricular defect are discussed.