The arterial switch operation has become the standard of surgical repair for total correction of complete transposition (concordant atrioventricular and discordant ventriculoarterial connections). A standard nomenclature for classification of the numerous anatomic relationships the coronary arteries may assume, however, has yet to be universally adopted. Inconsistent use of varied nomenclatures remain an obstacle to effective communication between physicians and surgeons. In this review we offer a unified nomenclature incorporating five important relationships: the configuration of the sinuses; their location; the configuration of the arterial orifices; the pattern of branching of the major coronary arteries; and the course of each artery.

The size of the coronary arteries parallels the ventricular mass, thus it may be abnormal in complete transposition beyond infancy and could be influenced by the arterial switch operation. To investigate this possibility, we measured the diameters of the right, left main trunk, anterior descending, and circumflex coronary arteries before and three to seven years (mean 4.8) after the arterial switch operation in 17 patients with a “normal” distribution of the coronary arteries (so-called Shaher type 1). The values were compared with 18 controls who had Kawasaki disease with no apparent coronary arterial disease. The right, left anterior descending, and circumflex arteries were smaller than control values before the operation. The post-/preoperative ratios of the diameter were 1.16±0.11 for the right coronary artery, 1.18±0.16 for the left main trunk, 1.20±0.18 for the left anterior descending artery, and 1.22±0.26 for the circumflex artery. There were no significant differences among these values. After surgery, the right coronary artery was larger, but the left coronary arteries were smaller in the patients than in the controls: 2.5±0.3 vs 2.0±0.2 mm for the right coronary artery; 2.4±0.3 vs 2.7±0.1 mm for the left main trunk; 1.9±0.2 vs 2.4±0.2 mm for the left anterior descending artery; 1.6±0.4 vs 2.2±0.5 mm for the circumflex artery, respectively. The posterior descending coronary artery originated from the right coronary artery in all patients. The total cross-sectional area of the right coronary, left anterior descending, and circumflex arteries was 9.7±2.4 mm2 in the patients, and 11.8±2.9 mm2 in the controls (p>O. 1), suggesting that the increased size of the right coronary artery compensates for the small left coronary arteries. We conclude that the arterial system in complete transposition, with a large right coronary artery and small left coronary system, remains smaller than normal even at midterm follow-up after anatomic repair despite normalization of left ventricular volume and muscle mass.

This study reviews the results of the surgical management of 154 cases of ruptured aneurysm of the sinus of Valsalva. Of the patients0 73% were male, with an average age of 28 years. An associated ventricular septal defect was found in 40% and 23% had aortic valvar regurgitation. The aneurysms originated from the right coronary sinus in 79% and from the non-coronary sinus in the remainders. The aneurysms ruptured into the right ventricle in 73%, into the right atrium in 27% and into the left ventricle in less than 1%. Operative mortality was 4.5%. Long-term follow-up was achieved in 80% of patients, with a mean duration of 5.7 years and a range from two months to 29 years. Preoperative aortic regurgitation and preoperative functional class (NYHA III or IV) were both predictive of a worse long-term outcome. The optimal surgical approach was closure of the distal end of the fistula by direct suture together with reinforcement of the aortic sinus with a Dacron patch.

Summary Between June 1988 and December 1992, six patients with dominant left and rudimentary right ventricles underwent orthoterminal correction with a modified Fontan operation in which the atriums were neoseptated using a flap constructed from the right atrial wali. Four patients had anomalous systemic venous connections. The operations were performed under direct caval cannulation, standard cardiopulmonary bypass, moderate hypothermia and cardioplegic arrest. After opening the right atrium with a longitudinal incision, the atrial septum was completely resected and the coronary sinus was cut back. The upper wall of the right atrial incision was brought down in such a way that the pulmonary venous atrium was drained into the dominant ventricle via the right-sided or common atrioventricular valve. This was followed by direct connection of the right atrium with its anterior wall reconstructed with pericardium to the pulmonary arteries. In one patient, a left superior caval vein draining to the left atrium was divided and anastomosed to left pulmonary artery. One patient died on the 12th postoperative day with pulmonary infection and sepsis, though he had no hemodynamic problem, and another died on the sixth day due to high pulmonary vascular resistance. The remaining four patients are progressing well at a mean of 23.4 months postoperatively with functional capacity of NYHA I-IT and sinus rhythm. Echocardiographic and angiocardiographic examinations during follow-up showed unobstructed pulmonary and systemic venous pathways in all. This modification of the Fontan operation seems a good alternative technique which creates a contractile left atrium with large enough dimensions and an unobstructed pathway for pulmonary venous flow.

We examined the effectiveness of the application of ice water to the face during metronome-controlled respiration (15 breaths per minute) for vagalstimulation. We also examined the importance of basal vagal tone and sympathovagal interaction in determining the individual response to the stimulation. Fifty-three boys, aged 12 and 13, were included in this study. Vagal tone and sympathovagal interaction were assessed by power spectral analysis of the variability of the RR interval (heart rate). Basal heart rate, high frequency power, and low-to-high frequency power ratios were 81 ± 13(58–110 beats/min), 791 ± 1061(56–4161 m.sec2) and 1.08±1.22 (0.04–4.85) during controlled respiration. After application of ice water, 23 children developed frequent nodal escape beats due to severe sinus bradycardia. Minimum heart rate, high frequency power, and low to high power ratios changed to 42± 12 (19–72 beats/min), 1890±1882 (211–7258 m.sec2) and 0.64±0.43 (0.12–1.46). The increased ratio of high frequency power, maximum decrement in heart rate, and its percent after stimulation were 5.44±5.62 (0.63–24.26), 39±14 (10–81 beats/mm) and 47±15 (16–81%), respectively. The increased ratio of high frequency power was correlated with basal logarithmic high frequency power (r=−0.60, p=O.0004). Maximum heart rate decrement was correlated with basal logarithmic high frequency power (r=−0.60, p=O.OO18) and low-to-high frequency power ratio (r=0.27, p=O.O4). We conclude that application of ice water to the faceduring controlled respiration induces powerful vagal stimulation and bradycardia similar to, or even greater than, facial immersion in cold water. There is wide variability of individual response which can be explained by the magnitude of absolute basal vagal tone.

We have investigated angiographically and histologically the vascular response to implantation of intravascular stents in normal puppies. Eight balloon-expandable stents were placed in six mongrel puppies (weight 6.4–10.5 kg) under anesthesia. Four stents were successfully placed in the pulmonary arteries and three in the aorta. In one case, the strut of the stent was hooked by a leaflet of the tricuspid valve at the time of placement and eventually had to be implanted in the inferior caval vein. Two puppies died, one from bleeding from the femoral artery and the other from thrombotic obstruction at the superior mesenteric artery, both after successful implantation. The former was due to repeat arterial puncture and the latter wasdue to failure to use aspirin. Angiographic studies showed satisfactory opacities of the branch arteries in all but one puppy. The diameter of the arteries prior to implantation was 7.5±1.5 mm. The diameters at the middle of the stents immediately after implantation and during the period of follow-up were 6.9±1.7mm and 6.9±2.1 mm, respectively. Histologic studies revealed that neogenic endothelial layers of 30 to 40 pm in thickness had formed over the struts of the stent without formation of thrombusthree weeks after implantation. Scanning electron microscopy revealed that the endothelial cells of the neoendothelial layer had almost identical characteristics to those of normal arteries. These results indicate that clinical application of this stent is feasible but that use of anticoagulants is mandatory during and after implantation in the treatment of selected patients with aortic coarctation or pulmonary arterial stenosis. Relative progression of stenosis may occur, however, and the inability for re-expansion is a major disadvantage of this stent.

The role of transesophageal echocardiography in the diagnosis and management of left-sided cardiac valvar disease in children and young adults was assessed in terms of whether additional information (over transthoracic echocardiography) could be obtained, and whether the added information contributed to the better management of the patients. Between January 1991 to August 1992, 27 consecutive patients were studied. Their age ranged from 2.5 to20 years (mean 12.7), with body weights ranging between 12 and 60 kg (mean 35.6). Twelve of the children suffered from atrioventricular valvar disease, 13 had predominant aortic valvar lesions and two had mixed valvar pathologies. Additional information was obtained by transesophageal over that of transthoracic echocardiography in 18 children (67%). Transesophageal echocardiography clarified the etiologies or mechanism of valvar regurgitation in eight and seven children with atrioventricular and aortic pathologies, respectively. The technique correctly diagnosed severe mitral regurgitation underestimated by conventional technique, detected a missed eccentric aortic regurgitant jet and convincingly excluded bacterial vegetations in one case each. The investigation contributed to the better management of three children (11%) by providing information that allowed the appropriate choice of therapy. Transesophageal echocardiography, therefore, is a useful technique for the diagnosis and management of valvar disease of the left heart in children and young adults.

Absent right atrioventricular connection, with the left atrium connected to a dominant left-sided morphologically right ventricle, is a rare situation. We report four cases, demonstrating the anatomical correlations and the clinical aspects needed to make the diagnosis. We also discuss the nomenclature of this important combination.

We discuss the management of 10 children with cardiac masses produced bytumors, thrombus or vegetations. The ages of the patients ranged from five days to nine years, only two being more than five years old. In all cases, the diagnosis was made by cross-sectional and Doppler echocardiography. Themasses were detected in the chambers of the right heart in five patients, in the left heart in four and on both sides in one patient. Five patients had predisposing factors. The clinical features were variable, but cardiac failure was the most common sign. All the patients underwent emergency surgery. Two patients died during the period of hospitalization and two in the late postoperative period. The six surviving patients are now asymptomatic.

Three patients aged five years, five years four months and 14 years with obstruction of the systemic venous baffle following the Mustard operation were treated with balloon dilation and implantation ofstents. Balloon dilation of the baffle obstruction was performed initially in the first two cases. In the third case, the obstruction was complete and was punctured with atranseptal needle via a 6 French transeptal sheath followed by a balloon dilation. in all three patients, a Palmaz stent (Johnson & Johnson, Summerville, New Jersey, USA) was loaded onto the balloon catheter and delivered into the stenotic area. There was complete relief of obstruction in allcases. The first case developed supraventricular tachycardia at the time ofcatheterization, the morning following implantation of the stent and thentwo weeks after that. There were no complications with catheterization and noshort-term side effects in the other cases. These cases illustrate the use of endovascular stents in the treatment of baffle obstruction.

Congenital pulmonary venous stenosis is a rare but important cause of pulmonary hypertension in infancy. The prognosis is uniformly poor, despite surgery and attempted balloon dilation of the narrowed veins. We performed intraoperative stenting of congenital pulmonary venous stenoses in two infants with initial clinical and hemodynamic improvement. Both patients subsequently died due to progressive pulmonary hypertension. Stenting of the pulmonary veins in this condition does not alter the natural history.

An 18-month-old black child became ill one month prior to admission to our center. She was shown to be Mantoux positive, with evidence of cardiomegaly on x-ray. Echocardiography revealed a progressive enlarging left ventricular aneurysm—a finding confirmed at cardiac catheterization. She underwent successful resection of the aneurysm. Histology proved it to be tuberculous in origin. To our knowledge, this is the youngest patient reported with such an aneurysm, and the first to have survived surgical correction.

We describe a rare type of atrioventricular septal defect with common atrioventricular orifice and malaligned atrial septum, the latter feature resulting in severe obstruction of the left atrial egress. Excision of the atrial septum and banding of the pulmonary trunk was performed at the age of thirteen months. Lung biopsy demonstrated marked medial hypertrophy with severe intimal fibrosis in the small pulmonary arteries and mild thickening of the pulmonary veins, considered contraindications for corrective surgery.

The period of adequate palliation from a Blalock-Taussig shunt may be limited by the development of stenosis of the shunt. Balloon dilatation oftenis unsuccessful due to recoil of the vessel. We report the case of a 22-year-old man with a stenosed Blalock-Taussig shunt who was successfully treated by the implantation of a balloon expandable stent.

A homogeneous opacity of the right upper lobe was found radiographically in a 15-month-old male child during investigation of an upper respiratory tract infection. Based on computerized tomography, the diagnosis was made of a solid tumor of the lung. At thoracotomy, dilated serpentine vessels were found on the surface of the right upper lobe, and the diagnosis of a large arteriovenous malformation was considered. Use of enhanced computed tomography without contrast and failure to note a reduced arterial oxygen saturation were two diagnostic pitfalls. The diagnosis of an arteriovenous malformation was confirmed by pulmonary angiography, and a right upper lobectomy was successfully performed.

A hydropic neonate, born at 28 weeks gestation, died 45 minutes after birth. Autopsy showed an abnormally long and tortuous arterial duct which wasoccluded by laminated thrombus. The right heart chambers and the left ventricle were dilated, but there was no other structural cardiac abnormality. Apart from the abnormal ductal morphology, there was no evident cause for the intrauterine thrombosis of the arterial duct, which appeared to be the sole cause of fatal heart failure.