Five infants under three months of age with tetralogy of Fallot and absence of the leaflets of the pulmonary valve were successfully corrected using a one-stage repair. All patients suffered from respiratory failure preoperatively and surgery was performed somewhat urgently. The age at surgery ranged from 26 to 72 days, with an average of 47±17 days. The surgical procedures involved four steps. First, we plicated the aneurysmally dilated central pulmonary arteries. Second, we closed the ventricular septal defect. We then inserted a handmade heterograft pericardial valve at the site of the pulmonary valve, and completed the reconstruction of the right ventricular outflow tract with a patch. All patients survived. Two of them were successfully weaned from ventilation at early (two and 11 days, respectively) periods after surgery, but the remaining three patients required artificial ventilation for one to two months. We conclude that one-stage repair is possible for this anomaly, but that plication of the pulmonary trunk is an essential technique todeliver patients in early infancy from respiratory failure.

To investigate the left ventricular systolic and diastolic function in patients with pulmonary atresia with intact ventricular septum without coronary-cardiac fistulas after a modified Blalock-Taussig shunt, 14 patients (age range 15 days-16.5 months, mean 4.03±5.6 months) and eight control subjects, matched for age, body surface area and heart rate were evaluated by cross-sectional and Doppler echocardiography. The follow-up interval after palliative procedures ranged from 12 days to 16.3 months (mean 3.67±5.6 months). Compared to controls, in the group of patients the ejection fraction was decreased (61±7% vs 68±5%, p=0.022) while the left ventricular end-diastolic volume indexed for body surface area was increased (72.7±10.8 cc/m2 46.1±12 cc/m2 p=0.0001) with normal values of left ventricular mass indexed for body surface area (67.88±20.9 g/m2 vs 76±10 g/m2 p=NS). Mass-to-volume ratio was lower in patients with pulmonary atresia (0.95±0.38 vs 1.24±0.3, p=0.031). The left ventricular shape index was increased in all patients with pulmonary atresia (1.27±0.26 vs 1±0.01, p=0.009). A significant inverse correlation was noted between the ejection fraction and follow-up (r=−0.71, p=0.04). as well as between the ejection fraction and shape index (r=−0.76, p=0.048). Moreover, the patients with pulmonary atresia had decreased E/A velocity ratio (0.65±0.16 vs 1.35±0.90, p=0.009), decreased normalized peak filling rate (4.16±0.13 sv/s vs 6.88±0.68 sv/s, p=0.0001), increased peak A velocity (0.95±0.17 m/s vs 0.51±0.16 m/s, p=0.0001) and prolonged isovolumic relaxation time (46±5.4 ms vs 34±6.2 ms, p=0.0001) and deceleration time (196.4±32.2 ms vs 116±21.4 ms, p=0.0001). There was a good correlation between the normalized peak filling rate and follow-up (r=−0.80, p=0.04). These data show a progressive compromise of the left ventricular systolic and diastolic function in patients with pulmonary atresia with intact ventricular septum without ventriculocoronary fistulas who had undergone systemic-to-pulmonary arterial shunting. Thus, an earlier biventricular or Fontan type procedure should be recommended.

Diffuse endocardial thickening in dilated hearts has been considered as a distinct pathological entity, usually being termed endocardial fibroelastosis. It is common in infants and children and rare in adults. Current opinion holds that the endocardial thickening is a non-specific reaction to stress in the ventricular wall. Our personal observations point to the most severe forms occurring in young infants. To determine the frequency and severity of endocardial fibroelastosis in children with dilated cardiomyopathy, we studied 21 necropsy hearts, both grossly and microscopically. Ages of the patients ranged from two to 175 months (mean 46.5, median 23 months). Using a graduated eyepiece, we measured the endocardial thickness in samples from three sites in the left ventricle and one from the right ventricle. The values were compared to those obtained from similar measurements of the endocardium in hearts from four children presenting with tetralogy of Fallot. All the dilated hearts presented some degree of endocardial thickening. The gross detection of fibroelastosis depended on the degree of thickening. There was a negative but linear correlation between age and the mean endocardial thickness (r=0.70; p>0.001). Our findings support the concept that endocardial cells have greater replicative and secretory potentials in young patients, and that endocardial fibroelastosis is a secondary phenomenon in dilated hearts.

Balloon dilation was attempted in seven symptomatic patients with congenital mitral stenosis aged 7.6±5.3 years. Of the five patients with typical mitral stenosis, four underwent successful dilation with decrease of the mean left atrial pressure and the gradient across the mitral valve and increases in the area of the valve of 127±43 and 85±16%, respectively, as demonstrated hemodynamically or by Doppler echocardiography. In the patient in whom the procedure was unsuccessful, a small child with recurrent pulmonary edema, death occurred during the procedure as a consequence of vasovagal reaction. In the other two patients, one with a parachute-like mitral valve and the other with Shone's syndrome, severe pulmonary hypertension and reversal of the shunt through a patent arterial duct, it was not possible to dilate the valve. In the patient with Shone's syndrome, nonetheless, palliation was achieved by balloon angioplasty of the aortic coarctation and by creation of an atrial septal defect. The patients undergoing dilation remained symptom-free during follow-up and mantained the increase in valvar area as judged by Doppler studies and, in one patient, also by cardiac catheterization. A residual atrial septal defect closed spontaneously in three patients. We conclude that balloon dilation of congenital mitral stenosis is an effective alternative to surgery in patients with typical mitral stenosis. Cross-sectional echocardiography plays an important role in defining valvar morphology and selection of patients. The procedure is not without risk, especially in very sick patients but satisfactory results are long-lasting.

In 38 children with aortic coarctation between the age of three weeks and 16 years, 44 balloon dilation procedures were attempted. At initial catheterization (n=38), the mean gradient dropped from 49.6±21.2 mm Hg prior to dilation, to 15.3±15.8 mm Hg after the procedure (p>0.05). Twenty-six patients were recatheterized 3–24 months after the initial procedure. The mean gradient remained low at 12.7±15.2 mm Hg (range 0–64). Six of the 26 (23%) patients required a second dilation because of significant recoarctation. This proved successful in four, but in two patients, the second dilation failed, and they were referred for elective surgery. Thus, good overall results were obtained in 24 of the 26 (92.3%) patients undergoing reinvestigation following balloon dilation. Complications included requirements for blood transfusion in 9/44 (20%), chest pain during balloon inflation in 3/44 (6.8%), rupture of the balloon in 3/44 (6.8%), and femoral arterial thrombectomy in 3/44 (6.8%). At a mean clinical follow-up of 21 months, 21% of the patients had a weaker femoral pulse on the side used for balloon dilation, while 21% had mild hypertension measured in the arms despite the absence of a significant gradient. One patient (2.6%) developed an aortic aneurysm as a complication of the procedure. We conclude that balloon dilation is a safe and effective procedure in the majority of the patients with native aortic coarctation.

Dissection of a pulmonary arterial aneurysm due to underlying pulmonary hypertension from congenital heart disease is uniformly fatal, but fortunately rare. Two such cases are presented, along with review of 24 other known cases published in the literature. Clinical presentation, guidelines tomanagement, and possible surgical intervention in the acutely dissecting patient are discussed.

The distribution of axial velocities in the pulmonary artery must be determined to describe accurately the flow in the pulmonary artery. Previous studies in our laboratory have demonstrated the complexity of the velocity profile in the pulmonary trunk and the branch pulmonary arteries with normal and altered pulmonary blood flow. These findings have demonstrated a skewed, parabolic mean velocity profile in the pulmonary trunk under normal hemodynamic conditions. To determine the effect of left-to-right intracardiac shunting on the distribution of axial velocities in the pulmonary artery, an animal model of atrial and ventricular shunting was used. In both models, the distribution of mean axial velocities remained skewed and parabolic with the higher mean velocities recorded toward the anterior wall.

Twenty cases of aortopulmonary window are reported with special reference to some unusual modes of presentation and hemodynamics. Patients over 15 years of age accounted for 35% of the group. This delayed presentation may relate to referral patterns and socioeconomic conditions prevalent in India. Curiously, this elderly subset had two modes of presentation—either as large left-to-right shunts with low pulmonary vascular resistance (71.4%), or as Eisenmenger's syndrome (28.6%). The reasons for the low pulmonary vascular resistance in the majority of these older patients remain unclear. The echocardiographic features of these defects can be missed in presence of significant associated cardiac lesions. A careful search should, therefore, be made for an additional aortopulmonary window in all cases with evidence of intercirculatory shunting.

We examined 25 heart specimens with aortopulmonary windows in order to review the variety of the lesion and its associated malformations. Unlike common arterial trunk, the aortopulmonary window involves a deficiency of the wall of the ascending aorta and that of the pulmonary trunk. The window was in proximal position in three specimens, intermediate position in three specimens, distal position in 16 specimens and was confluent in three specimens. The size of the window varied from 27 to 100% of the total length of the pulmonary trunk but size did not bear any apparent relationship to the position of the defect. The shape of the window was tunnel-like in one case. Of the 16 specimens with distal windows, the orifice of the right pulmonary artery arose from the aorta in seven specimens and was overriding the plane of the window in one specimen. The window occurred as an isolated lesion in four specimens. It was associated with interruption of the aortic arch at the isthmus in eight specimens and between the left carotid and left subclavian arteries in three specimens. A further three specimens had isthmal stenosis. Among the other associated defects were complete transposition (two specimens), tetralogy of Fallot (one specimen), and double outlet right ventricle (one specimen). A ventricular septal defect was present in six of the 25 specimens. The associated lesions found with aortopulmonary window are seldom encountered with common arterial trunk, suggesting these two arterial lesions have different pathogenesis and are not variants of the same entity.

Transesophageal echocardiography and magnetic resonance imaging have proved useful as noninvasive imaging modalities in postoperative patients with congenital heart disease, or patients with suboptimal transthoracic echocardiograms. Each modality has specific advantages. When both are of equal efficacy, magnetic resonance imaging is recommended because it is less invasive and more comfortable for the patient.

Cardiac and extracardiac anomalies tend to follow characteristic patterns when there is an isomeric arrangement of the heart and lungs. We present a case diagnosed correctly during prenatal life as having isomerism of the left atrial appendages in which some of the associated features were most unexpected, namely absence of the spleen and obstructed totally anomalous pulmonary venous connection. This association proved misleading for us in the management of the patient. We found it difficult to believe our findings in the light of our past knowledge, and this resulted in a delay in the referral for surgery. The final outcome was unfortunately fatal. In the management of isomerism of any type, therefore, a wide spectrum of combinations and “crossover” of cardiac anomalies must be anticipated, emphasizing the need to assess individually the arrangement of the different organs of the body.

Coarctations of the aortic arch are rare, and when found, they are often associated with other complex congenital heart disease. An adolescent female with multiple coarctations of the aortic arch and ventricular septal defects underwent complete repair, entailing replacement of the aortic arch with a homograft and closure of the ventricular septal defects. The postoperative course was complicated by a tear in the homograft, possibly related to sepsis. After its resolution, her hemodynamic results remain excellent.

We describe an infant with complex congenital heart disease, including double orifice of the mitral valve associated with complex subaortic stenosis. Transthoracic and intraoperative transesophageal echocardiography were used to define three distinct anatomic abnormalities, all potentially contributing to the subaortic obstruction. Stepwise surgical repair with transesophageal echocardiographic monitoring of hemodynamic function ensured optimal initial surgical results. Overwhelming postoperative complications ensued, however, and the patient died. Postmortem findings are also presented.

Communication of the coronary sinus with the left atrium is an unusual anomaly. Two cases of tricuspid atresia are described where systemic desaturation developed following a modified Fontan procedure. In both, investigation demonstrated a communication between the coronary sinus and the left atrium. Transcatheter closure of these communications was achieved with a ductal device in one case and coil embolization in the other. Following occlusion and elimination of the right-to-left shunt, both patients became fully saturated in room air.

Accessory atrioventricular valvar tissue is uncommon and, on occasion, has been identified as a cause of ventricular outflow obstruction. Accessory tricuspid valvar tissue has been reported to cause subpulmonary obstruction but infrequently has accessory tissue arising from the mitral valve been associated with obstruction. This paper reports two cases of subvalvar obstruction; the first in association with a ventricular septal defect causing subaortic obstruction and the other in association with congenitally corrected transposition and a ventricular septal defect, causing subpulmonary obstruction.

A two-month-old boy, with complex pulmonary atresia, became increasingly cyanosed 10 days after a modified 5 mm right Blalock-Taussig shunt. There was no shunt flow on auscultation or Doppler echocardiography. Despite anticoagulation with heparin, the shunt remained occluded. After demonstration of the occluded shunt at angiography, a guide wire was easily passed through the occlusion and into the pulmonary artery. Balloon dilation was then successfully accomplished with a 6 mm diameter angioplasty balloon resulting in a patent shunt. The shunt was still widely patent at cardiac catheterization six months later.

Isolation of the right subclavian artery from the aortic arch is a rare malformation. When isolated, the artery originates from the right pulmonary artery via a right-sided arterial duct, which may be patent or closed at the time of presentation. Intracardiac or other anomalies of the great vessels are commonly associated with this lesion. We present a case of isolation of the right subclavian artery which was initially diagnosed as persistent patency of a left-sided arterial duct. Surgical correction was eventually achieved by disconnecting the right subclavian artery from the right pulmonary artery and translocating it to the ascending aorta.

A rare association of ‘absent pulmonary valve’ syndrome, midmuscular ventricular septal defect and aneurysmatic dilation of the ascending aorta without pathology of the aortic valve is described in a 10-month-old infant. The diagnosis was established by echocardiography and angiography.

Summary Absence of the arterial duct has been reported in association with tetralogy of Fallot, with pulmonary stenosis or atresia, other complex cyanotic congenital heart diseases, and anomalies of the aortic arch but not, as far as we are aware, with tricuspid atresia. We report the clinical, surgical, and autopsy findings of a patient with tricuspid atresia whose initial clinical course was influenced by the absence of thearterial duct.