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Recent experimental studies and clinical application of deep hypothermia for cardiovascular surgery are reviewed. At most institutions, surface hypothermia alone or in combination with limited cardiopulmonary bypass has been employed. Circulatory dynamics were well maintained following prolonged cardiac arrest at 20°C. Some degree of acidosis usually developed after the arrest period but was gradually corrected during rewarming. Total circulatory occlusion could be maintained for at least one hour at 20°C. without evidence of cerebral damage in infants. Many complex congenital cardiac anomalies, including transposition of the great arteries, total anomalous pulmonary venous return, ventricular septal defect, and tetralogy of Fallot, have been successfully corrected in the first few weeks of life. Less encouraging results have been achieved in patients with infradiaphragmatic total anomalous pulmonary venous return, complete atrioventricular canal, or pulmonary atresia.

Five neonates with congenital tricuspid incompetence due to severe tethering of the tricuspid valve to the right ventricle by abnormal chordal and papillary muscle attachments are described. The abnormality was called tricuspid valvular dysplasia (TVD) if the basal insertion of the valve was normal and the Ebstein malformation if it was displaced into the sinus portion of the ventricle. In the two infants with isolated TVD and severe tricuspid regurgitation the functional obstruction to right ventricular outflow (ORVO) produced by the high perinatal pulmonary vascular resistance (PVR) made the exclusion of pulmonary atresia difficult, despite selective right ventricular angiocardiography. In three infants the tricuspid valvular abnormality was associated with organic ORVO; pulmonary atresia in two and critical pulmonary valve stenosis in one. In the two infants with pulmonary atresia and intact ventricular septum (IVS) the severe tricuspid incompetence produced a clinical, radiological and hemodynamic profile which was clearly different from that usually seen in infants with pulmonary atresia and IVS and a normal right ventricular cavity (type 2 of Greenwold).

Superior vena cava-right pulmonary artery anastomosis has been used to palliate cyanotic congenital heart disease in 42 patients with the following anomalies: tricuspid atresia, 19 cases; transposition of the great vessels, 11; pulmonary atresia, 6; truncus arteriosus, 3; Epstein’s malformation, 2; and atrioventricular canal with pulmonary stenosis, 1 case. Twelve of 13 operative deaths occurred in the 24 infants younger than 6 months of age. Five of 6 infants less than 1 month old died after the operation. Six late deaths were distributed among all age groups and all cardiac lesions. Although survivors were improved clinically, there is a gradual progression of cyanosis and rise of the hemoglobin level. It is clear that cava-pulmonary anastomosis should not be done in the first month of life. An ascending aorta-right pulmonary artery shunt is preferable, even in the first 6 months if the pulmonary artery is small. The cava-pulmonary anastomosis enlarges as the child grows, but increasing venous collateral may reduce its effectiveness. When complemented with a systemic-pulmonary shunt it has the potential for long-term palliation of uncorrectable congenital lesions.

The early results and late developments are presented of a consecutive series of 36 patients who underwent anastomosis of the ascending aorta to right pulmonary artery during a six-year period for a variety of congenital malformations associated with cyanosis and severe pulmonary stenosis or atresia. Twenty-six were infants under one year of age, and 16 of these were under one month of age at the time of operation. The early operative mortality of 22% was related to the severity of the anomaly and the young age of the patient. There were no early deaths in those aged three months or more. If the anastomosis was patent, symptomatic relief was good. However, the long-term mortality was 53%. Except for one baby who died of an associated congenital anomaly, the deaths were related to some consequence of the Waterston anastomosis that was unforeseen at the time of its employment. These included obliteration of the anastomosis, kinking of the anastomosis with partial or complete interruption of continuity between right and left pulmonary arteries, obliteration of the right pulmonary artery, progressive severe obstruction to right ventricular outflow and decreased flow to the left lung, as well as the complications of an excessive shunt with cardiac failure and pulmonary vascular obstructive disease. One or more subsequent palliative operations were required in nine symptomatic patients. Of the ten children with tetralogy of Fallot who underwent later open-heart repair, four died because of the difficulty in takedown of the Waterston and relief of the acquired stenosis or atresia of the right pulmonary artery and of the right ventricular outflow tract and hypoplasia of the left pulmonary artery. This mortality rate of 40% in these tetralogies contrasts with a concurrent mortality rate of less than 5% in a consecutive series of 50 patients with a tetralogy who had not had a previous Waterston procedure. We believe that this form of palliation should be reserved for those symptomatic, cyanotic newborns who are candidates for palliative rather than reparative surgery and who are not suitable for a Blalock-Taussig or Potts anastomosis.

PubMed ID: 4420177, The ECGs of 473 infants under the age of 3 months who were referred to a paediatric cardiological unit were analysed; 47 (10%) of the ECGs showed a superior axis (dominantly negative deflection S wave, in lead aVF). Of these, the majority of noncyanosed patients with plethora on chest x-ray proved to have either an atrioventricular canal defect or a large ventricular septal defect. When cyanosis and pulmonary plethora on x-ray were present, tricuspid atresia with increased pulmonary flow (types Ic or IIc) or d-transposition with ventricular septal defect accounted for most cases. With cyanosis and pulmonary oligaemia on x-ray, tricuspid atresia (types la and b) or pulmonary atresia with ventricular septal defect accounted for all cases. Finally, 2 patients with superior axis presenting in a shocked condition were found to exhibit the hypoplastic left heart syndrome. Recognition of superior axis in the ECG provides a useful diagnostic aid in congenital heart disease in early infancy.

Accurate definition of the site and length of the atretic segment in the patient with pulmonary atresia and intact ventricular septum is of prognostic and surgical significance. A double catheter technique was developed to allow precise localization of the site of atresia, whether valvular, infundibular, or both. Simultaneous opacification of the pulmonary artery by way of the ductus arteriosus or systemic to pulmonary arterial anastomosis by one catheter and the right ventricle by the second catheter was accomplished in five patients with pulmonary atresia, severe hypoplasia of the right ventricle and intact ventricular septum. The patients were 2 days to 17 months of age and weighed 3,200 to 9,200 g at the time the technique was applied. In these patients, the site of atresia proved to be valvular in one, and both valvular and infundibular in four. The double catheter technique is also applicable to patients with ventricular septal defect and acquired or congenital pulmonary atresia who have had palliative shunts, and to patients with complex transpositions and pulmonary atresia.

A new multiple crystal echocardiographic system was used to assess cross-sectional cardiac anatomy in real time in fifty infants and children with cyanotic heart disease, and the findings were compared to normals. Four standard transducer positions were employed to evaluate sagittal and transverse cardiac cross-sections. Studies in patients with tetralogy of Fallot demonstrated an enlarged aorta overriding the septum with preservation of mitral-aortic continuity. In the absence of pulmonary atresia the main pulmonary artery could be identified in all of these patients. The diagnosis of d -transposition of the great vessels was established by viewing great vessel orientation in transverse section at the second intercostal space and observing a rightward and anteriorly placed great artery. In sagittal projections a retrosternal aorta and mitral-pulmonic continuity was observed. In one patient with d -transposition, apposition in systole of the ventricular septum and anterior leaflet of the mitral valve was identified as the cause of left ventricular outflow tract obstruction. Descriptions are provided of the findings in patients with l -transposition, double outlet right ventricle, and truncus arteriosus. The new multiscan method allows a substantially more precise determination of intra- and extra-cardiac spatial relationships than single crystal techniques. Multiscan echocardiography is safe, widely applicable, and provides clinically important information to assist in the diagnosis and management of infants and children with cyanotic heart disease.

Pulmonary atresia ischaracterized bydevelopmental abnormalities ofthepulmonary valve or pulmonary arterial tree, withcomplete obstruction toblood flowbythenormal route.We recognize three maintypes, basedonembryology, anatomy, andsuitability forsurgical treatment. Pulmonary atresia asanelement ofamorecomplex lesion such astransposition ofthegreat vessels ortricuspid atresia isclassified undertheprimary abnormality. (1)Pulmonary ValveFusion withIntact Interventricular Septum: embryologically thisisthe extreme formofsimple pulmonary valve stenosis, withcomplete fusion ofthepulmonary valve cusps. Aninteratrial communication ispresent, usually a patent foramen ovale, andbloodreaches thepulmonary arterial tree distal totheobstructed pulmonaryvalve bywayofapatent ductus arteriosus. (2)Extreme Fallot's Tetralogy withPulmonary Atresia: basically theanatomy isthat ofthetetralogy ofFallot, withalarge ventricular septal defect and dextroposed aorta, butthepulmonary valve and sometimes themainpulmonary artery aswell are poorly developed andtotally obstructed. Right andleft branches ofthepulmonary artery arepresent andcommunicate witheachother across themidline.Blood reaches these vessels viaapatent ductus arteriosus orbronchial collateral vessels orboth. Taussig (1947) originally confined this termtocases withafunctioning patent ductus arteriosus, butwe extend ittothose withbronchial collateral vessels as anadditional oralternative source ofbloodsupply to thebranches ofthepulmonary artery.

SUMMARY A case of pulmonary atresia with anastomotic vessels connecting the right ventricle with the aorta has been described. Angiocardiographic films suggest that the anastomotic channels are right and left coronary arteries.

Fifty cases of pulmonary atresia with intact ventricular septum were reviewed. The patients were divided into two groups depending on the size of the right ventricle. Clinical, roentgenographic, and electrocardiographic findings in each group are discussed. The most reliable method for determining the size of the right ventricle is selective angiography with injection of dye into the right ventricle. Up until 1967, with or without having surgery, patients of both groups died. Since then emergency cardiac catheterization and balloon septostomy followed by operative creation of a Potts' anastomosis and ligation of patent ductus arteriosus has improved results in patients with a small right ventricle.

A 3 month old girl with cyanosis was found to have isolated right ventricular hypoplasia by cardiac catheterization and angiography. On physical examination, no murmur was heard and a single second heart sound was noted. The electrocardiogram revealed a frontal plane ÂQRS of 40 °, left atrial enlargement and left ventricular preponderance for age. Chest x-ray examination was not remarkable and probably normal. At cardiac catheterization, a large right to left shunt at the atrial level was demonstrated in spite of normal right-sided pressures. Angiograms revealed a diminutive right ventricular chamber which emptied into normal-sized pulmonary arteries. Intramyocardial contrast material was present after the injection, but no morbidity resulted; however, right ventricular injection of contrast material may be more hazardous in patients with isolated right ventricular hypoplasia. The clinical findings in reported cases so vary that, at present, cardiac catheterization is necessary for definitive diagnosis of this rare entity. The clinical findings in isolated right ventricular hypoplasia may mimic those of tricuspid atresia or type I pulmonary atresia and should be included in the differential diagnosis of any patient with cyanosis and left ventricular enlargement or preponderance.

Two cases of cyanotic congenital heart disease are reported in which an unusual mechanism seems to have operated to cause sudden death. In one patient the major lesions were pulmonary atresia and tricuspid stenosis. In the second patient the chief anomalies were aortic and mitral stenosis. In both instances thrombi developed just above the anomalous semilunar valves and ultimately grew large enough to obstruct vital circulatory pathways and produce a sudden fatal termination. As surgical procedures are developed for the alleviation of congenital anomalies, it is possible that care will have to be taken to avoid the creation of cul-de-sacs in which thrombi may form and lead to serious consequences at a later date.

Angiographic examination of 7 patients with a systemic arterial supply of the lungs with malformation of the pulmonary circulation and of 2 patients with bronchopulmonary anastomoses and pulmonary atresia with ventricular septal defect illustrates the value of selective systemic angiography in such conditions, especially for demonstrating the origin and course of the anomalous arteries and their venous drainage.

Thirty-nine autopsy cases of Type i pulmonary atresia with an intact ventricular septum have been reviewed. Clinical correlation was available in 19 cases; catheterization and angiocardiographic data were included in 7. This complex is associated with an extremely thickwalled but minute capacity right ventricular chamber which is generally not amenable to correction by pulmonary valvotomy. Twelve patients underwent attempts at surgical palliation, including valvotomy and various systemicpulmonary artery anastomotic procedures. Only 3 patients survived the immediate postoperative period. All 3 had undergone aortopulmonary anastomosis, surviving, respectively, two months, fourteen months and three years postoperatively. Since hypoplasia of the right ventricular cavity is a major factor in limiting the surgical approach in this lesion, particular attention was given to the correlation of the electrocardiogram with right ventricular anatomy. Of 13 patients for whom 12-lead scalar electrocardiograms were available, 9 had left ventricular hypertrophy or left ventricular preponderance (absence of the expected right ventricular preponderance for age). The remaining 4 infants, all with equally small right ventricular chambers, had patterns of right ventricular or combined ventricular hypertrophy with varying degrees of right bundle branch block. In addition, an age-dependent progression in right ventricular potentials and occurrence of right bundle branch block were suggested from a review of individual and serial electrocardiograms. Nine infants with electrocardiographic left ventricular hypertrophy or preponderance were all under 3 months of age. In contrast, 3 of 4 infants with records of right ventricular or combined ventricular hypertrophy were 3 months or older. Thus, when a right ventricular or combined ventricular pattern was noted in Type i atresia, the electrocardiogram could not be relied upon to accurately mirror right ventricular chamber size. The results of operation for this malformation, regardless of the procedure attempted, are discouraging, particularly when contrasted with those for tetralogy with pulmonary atresia and tricuspid atresia at comparable ages. The factors that contribute to the high operative mortality are not adequately understood.

Two patients with tetralogy of Fallot with previous functioning shunt developed obstruction of the right ventricular outflow tract. Preoperative diagnosis and total corrective surgery were successfully carried out. The acquired obstructions were clearly demonstrated by the series of angiocardiograms. Pulmonary atresia may be suspected clinically by disappearance of the systolic murmur. Acquired obstruction of the right ventricular outflow tract appears to be associated with angiocardiographic evidence of increase in the degree of overriding of the aorta. Prompt correction of the defect should be considered after angiocardiographic confirmation of the acquired atresia of the right ventricular outflow tract in such patients.

1. Babies with pulmonary atresia and intact ventricular septum rarely survive for more than a few weeks after birth. Prompt diagnosis and recent advances in surgical treatment now offer greatly improved possibilities for long term survival.2. Emergency angiocardiography is necessary for precise preoperative diagnosis.a. Venous or right atrial angiocardiograms are generally inadequate for accurate diagnosis.b. Selective right ventricular angiocardiography best shows the size and position of the right ventricle, the pulmonary valvular atresia and the intact ventricular septum, but poorly demonstrates the left heart and the lung vessels. Tricuspid atresia is ruled out.c. Selective left ventricular angiocardiography shows the left heart, aorta and pulmonary blood supply as well as the distal surface of the atretic pulmonary valve. The right coronary artery position permits an estimate of right ventricular size.d. A combination of both selective right and left ventricular angiocardiograms is ideal. Either exam...

A clinical, radiological and anatomical description of a case of combined tricuspid and pulmonary atresia with associated underdevelopment of the right ventricle and left juxtaposition of auricles.

A recent review of the literature found 123 cases of pulmonary atresia with intact interventricular septum. This report adds three more cases. It is suggested that immediate diagnostic studies followed by surgery may improve the chances of survival in a condition which has had a mortality rate approaching 100 per cent. The patients reported here are now 2 years 4 months, 2 years 2 months, and 1 year 4 months of age and doing well.

A case of congenital diverticulum of the left ventricle associated with a ventricular septal defect and severe hypoplasia of the right ventricle without tricuspid or pulmonary atresia is described and the relevant literature is reviewed. The distinction between true muscular diverticulae and congenital apical aneurysms is made, the latter term being preferred for similar abnormalities described as fibrous diverticulae or congenital epicardial cysts. Rupture is rare in true muscular diverticulae and the high pressure recorded in our case is thought to have been caused by catheter entrapment.

Based upon a series of 57 necropsy cases and upon the literature, it was found that there are two basic types of common aorticopulmonary (A-P) trunk: those with a ventricular septal defect, and those without. The latter is exceedingly rare and did not occur in this series. Truncus with a ventricular septal defect was found to consist of the following: (1) absence of the distal portion of the pulmonary infundibulum (very similar to tetralogy of Fallot with pulmonary atresia); (2) partial or complete absence of the pulmonary valve; (3) partial or complete absence of the aorticopulmonary septum; and (4) arterial arches 4 and 6 varied inversely in their development (e.g., well developed aortic arch with absent ductus arteriosus, or interrupted aortic arch with a large patent ductus arteriosus). Thus, there appeared to be no such thing as “true” persistent truncus arteriosus in the timehonored sense of persistence of an undivided conotruncal channel. While it is correct that the truncus is undivided (the aorticopulmonary septum being partially or totally absent), at the level of the conus the anomaly is not a failure of septation but absence of the entire distal portion of the pulmonary infundibulum (septum and free wall). Hence, at the level of the conus, common trunk is not a large infundibular septal defect but an extreme tetralogy of Fallot. At the distal conus, nothing has persisted. When the aortic arch was present, there was a high incidence of right aortic arch (27%) similar to tetralogy. The truncal valve basically was the aortic valve, explaining why it usually was tricuspid (67%). When some pulmonary leaflet tissue persisted, made possible by absence of the aorticopulmonary septum at the semilunar valve level, the truncal valve then was quadricuspid (24%). Failure of leaflet separation either in a basically tricuspid or quadricuspid valve occasionally resulted in a bicuspid truncal valve (7%). Truncus is not a form of transposition of the great arteries. Mitral-aortic fibrous continuity was present in all cases. The ventricular septal defect is not primarily “membranous.” It is due to absence of the distal pulmonary infundibulum. The coexistence of single ventricle and common aorticopulmonary trunk has been considerably overestimated. This combination did not occur in this series and is extremely rare (?non-existent). A revised and simplified classification of truncus is proposed.

Fifty patients with congenital pulmonary atresia and ventricular septal defect are separated into three relatively distinct physiologic, clinical and therapeutic categories, according to the amount of flow of pulmonary blood through aorticopulmonary communications. Clinical characteristics of the entire group are summarized, and additional findings that permitted separation of patients into one of three therapeutic groups are analyzed. Twenty-two infants with minimal aorticopulmonary communications had maximal hypoxia, and only those in whom successful palliative surgery was accomplished survived the first year of life. In 13 patients pulmonary flow was adequate during infancy but thereafter became sufficiently limited to require surgery. Fifteen patients had adequate pulmonary blood flow in infancy or childhood, and in 2 of these increased pulmonary flow through collaterals led to heart failure in early infancy. None of these 15 patients has required palliative surgery so far. The methods and results of palliative surgery in 28 infants and children are presented. An ascending aorta to right pulmonary artery shunt is the procedure of choice in the very young. Other types of systemic to pulmonary artery anastomosis may be utilized in older children. Successful total correction of patients with congenital pulmonary atresia and ventricular septal defect requires the presence of pulmonary arteries of adequate size. Half our patients had pulmonary arteries at least 5 mm. in diameter and therefore could be considered eligible for complete repair. Palliative surgery in infancy and early childhood, in addition to alleviating the catastrophic consequences of anoxia, also may promote sufficient growth of the pulmonary arteries to make definitive surgery more feasible at a later age.

This article describes a surgical technique for correction of cardiac anomalies in infancy, which utilizes surface cooling to low temperatures, intracardiac repair during a 1-hr circulatory arrest period, and rapid rewarming by total body perfusion. The metabolic changes occurring with this technique and the postoperative management are detailed. There were 14 hospital deaths among the 67 infants with correctable lesions operated upon in the first year of life (21%). For infants under 1 mo, the mortality was 50%, for those aged 1–6 mo it was 22%, and for those aged 7–12 mo it was 0%. Mortality was highest in two complex conditions, namely total A-V canal and transposition of the great vessels when combined with a a large ventricular septal defect (with or without coarctation of the aorta). If these two conditions were excluded, hospital mortality fell to 9%, and the apparent increase in risk with decrease in age became much less important. Only one infant suffered cerebral damage from the circulatory arrest period, and this was minor. An important feature of the technique has been the virtual absence of major pulmonary problems. In view of the results achieved, it is considered that one-stage correction is preferable to palliation, regardless of age, in almost all correctable conditions. In particular, this is so in large ventricular septal defect, total anomalous pulmonary venous connection, partial A-V canal, truncus arteriosus, and pulmonary atresia with large ventricular septal defect, tetralogy of Fallot, and transposition of the great vessels.

The records of 15 infants with pulmonary atresia and 19 infants with critical pulmonic stenosis and intact ventricular septum, all under 1 year of age, have been reviewed. Symptoms started earlier and were more severe in the group with pulmonary atresia. All patients showed arterial unsaturation, and many had congestive heart failure The electrocardiogram was the most helpful tool in differentiating pulmonary atresia from pulmonic stenosis with intact ventricular septum. The presence of P pulmonale, significant right axis deviation, and right ventricular hypertrophy favors pulmonic stenosis. At cardiac catheterization, patients belonging to both groups showed right ventricular hypertension of significant degree, but this was more marked in infants with pulmonic stenosis. Results of surgery for pulmonary atresia with intact ventricular septum, by a variety of methods, have been uniformly poor up to the present. Direct attack on the pulmonary outflow tract, when possible, may offer more promise for the future. Pulmonary valvotomy is the operation of choice for pulmonic stenosis and the results are good.

A three and one-half month old infant with corrected transposition of the great vessels, pulmonary atresia, and intact ventricular septum is presented. Cardiac catheterization and angiocardiography disclosed a small blind right-sided pulmonary (anatomic left) ventricle with systolic pressure exceeding systemic levels. The chamber communicates with the coronary arterial system via transmyocardial sinusoids. To our knowledge, this is the first report of this syndrome complex diagnosed during life.

Hypoplasia of the right ventricle, unassociated with severe pulmonary or tricuspid valvar malformations, is a primary congenital abnormality in which the trabeculated sinus portion of the ventricle fails to develop. An atrial septal defect or a stretched foramen ovale serves as an escape valve. The clinical, hemodynamic, and angiocardiographic features of this rare type of cyanotic heart disease are described in two children and reviewed in 13 previously reported cases. The clinical spectrum varies considerably in accordance with the degree of hypoplasia. Severe forms of hypoplasia, usually presenting in infancy, closely resemble tricuspid or pulmonary atresia. The electrocardiogram in hypoplasia, which shows right atrial enlargement and absence of right ventricular electrical forces, may be indistinguishable from an electrocardiogram in atresia. Lesser degrees of hypoplasia should be differentiated from Ebstein's malformation and anomalies of the systemic venous return. Both our patients, who had only mild underdevelopment of the right ventricle, markedly improved after surgical closure of the atrial septal defect. It is proposed that in mild forms of hypoplasia temporary occlusion of the atrial septal defect by means of a balloon catheter should be used in the preoperative assessment of right ventricular function.

A third case of congenital marked hypoplasia of the right ventricular myocardium (Uhl's anomaly) associated with pulmonary valvular atresia and absent tricuspid leaflets is reported. The anatomic differences between this disease, hypoplasia of the right ventricle and Ebstein's anomaly are discussed. During the neonatal period the clinical diagnosis is difficult. Malformations like tricuspid atresia, pulmonary atresia with intact ventricular septum and Ebstein's anomaly can all give almost the same clinical picture. The prognosis of the complete form of Uhl's anomaly remains poor.

An unusual malformation of the heart in a newborn infant is described, consisting of persistent venous valves in the right atrium, hypoplasia of the tricuspid valve, pulmonary atresia, and multiple coronary artery-ventricular communications with the right ventricle. Coronary flow to the right heart was shunted through the right ventricle, and focal necrosis was seen in the right ventricular myocardium. The pertinent literature is reviewed and the pathogenesis of the malformation is discussed.

A LTHOUGH AN UNCOMMON MALFORMATION, pulmonary atresia with intact ventricular septum requires more attention because of the recent availability of surgical procedures. Without prompt diagnosis and operation, the lesion is always fatal, frequently in the neonatal period.1 In this entity, fusion of the pulmonary valve cusps produces complete obstruction of the right ventricular outflow tract. A right-to-left shunt is essential for survival and this occurs at the atria1 level. Perfusion of the lungs is accomplished through a persistent ductus arteriosus.

When the pulmonary valve is atretic and the ventricular septum intact, the right ventricle usually consists of a small chamber with a very thick wall capable of developing high pressure. This pathologic picture is associated with clinical findings similar to those in tricuspid atresia—cyanosis, decreased pulmonary flow, left ventricular preponderance on the electrocardiogram, and early death. The diagnosis may be confirmed by heart catheterization and selective angiocardiography with injection into the right ventricle, but the risk is great. Surgery has never been successful in the past; but because of the equally hopeless prognosis on medical treatment, attempts should continue to be made. Anastomosis of the superior vena cava to the right pulmonary artery appears to offer hope of success in the future.

Isolated atresia of the pulmonary valve is a rare congenital malformation. Two types are recognized; one in which the right ventricle is small and its cavity slit-like in character, and another in which this ventricle is large. Pulmonary blood flow is dependent upon a patent ductus. If the lives of the infants are to be salvaged, the lesion must be diagnosed, and operation performed before the ductus closes. Pulmonary valvotomy is recommended when a large right ventricle is present. When this ventricle is small, a palliative superior vena cava to pulmonary artery anastomosis is advocated. Seven infants with pulmonary atresia are reported. Three died before operation could be undertaken and four had pulmonary valvotomy performed. Of these four, one died postoperatively and three survived to be discharged from hospital. One of these died at six months with reclosure of the pulmonary valve orifice; one died following a repeat operation; and the lone survivor at eighteen months continues to do well, but has some residual pulmonary stenosis.

The usual causes of a large right to left shunt at the atrial level are tricuspid or pulmonary atresia, severe pulmonary stenosis, Ebstein's anomaly or a large atrial septal defect with pulmonary hypertension. We have recently evaluated a patient with a large right atrial membrane which subdivided the right atrium into 2 chambers with the venae cavae and foramen ovale on one side of the web and the coronary sinus and a small tricuspid valve on the other. The presence of this membrane was demonstrated preoperatively by cineangiograms, and it was successfully removed at operation. This unusual entity, cor triatriatum dexter, is discussed and the literature reviewed.

External scintillation scanning of intravenously administered 131 I-labeled macro-aggregates of human serum albumin ( 131 I-MAA) was employed to evaluate the distribution of pulmonary arterial blood flow in 61 normal subjects and in 100 patients with various congenital cardiovascular malformations. Intra-aortic administration of 131 I-MAA demonstrated that blood flow through subclavian-pulmonary artery anastomoses is directed principally to the lung on the side of the anastomosis; the relative concentration of 131 I-MAA in each lung after intravenous injection provided an index of the patency of the anastomosis or of the development of pulmonary atresia or pulmonary hypertension. In contrast to the findings in patients with a patent subclavian-pulmonary shunt, scans obtained from patients with a patent ductus arteriosus did not reveal a separation of the systemic arterial and systemic venous inflows to the lungs. The patency of superior vena caval-right pulmonary arterial anastomosis could be assessed after injection of 131 I-MAA into an upper-extremity vein. Anomalies characterized by increased pulmonary blood flow or elevated pulmonary arterial pressures, or both, increased the ratio of pulmonary blood flow in the lung apices relative to that in the dependent lung zones. Anomalies characterized by elevated pulmonary venous pressure, such as cor triatriatum and mitral regurgitation, were readily detected by demonstrating both a decrease in blood flow to the lung bases as well as an increase to the apices. Thus, in patients with known pulmonary arterial hypertension (mean pressure, >30 mm Hg) the ratio of upper to lower zone blood flow was always significantly higher if the arterial hypertension was accompanied by venous hypertension. For this reason, lung scans facilitated the screening of patients with pulmonary arterial hypertension for surgically correctable lesions such as cor triatriatum and mitral stenosis. The method described is technically simple, without risk, easily applicable to large numbers of patients, and provides clinically important information concerning many forms of congenital heart disease.

A case of “absent inferior vena cava” is described, associated with a dextroposed biloculate heart with pulmonary atresia and patent ductus arteriosus. Seven additional cases, with heart findings, from the literature are summarized, and attention is drawn to the diagnostic implications of this anomaly. This anomaly of the inferior vena cava is best described as anomalous inferior vena cava with azygous drainage.

Four patients with tetralogy of Fallot syndrome, whose physical and x-ray findings were distinctly unusual, are presented in this report. All these patients were demonstrated to have functional or anatomic atresia of one of the main pulmonary arteries, in addition to the characteristic features of the tetralogy of Fallot. Diagnostic criteria, including angiocardiograms, are presented, and the therapeutic implications are discussed.