Atresia of the left atrioventricular valve with patency of the aorta: anatomico-functional analysis of 23 patients.

We analyzed the findings in 23 patients with atresia of the left atrioventricular valve and a patent aorta seen in the period from January 1980 to July 1989. Having divided the cases according to the anatomical findings, we made a subsequent analysis of the clinical and surgical results with the aim of establishing the management most likely to diminish risks, still high, in treatment of this complex anomaly. From the anatomical viewpoint, three variants were observed. In the first, made up of 15 cases, there was absence of the left atrioventricular connexion. The characteristic finding in the second group, with five cases, was an imperforate left atrioventricular valve in the setting of concordant atrioventricular connexions. The third group, of these cases, was dominated by the presence of isomerism of the atrial appendages, both appendages being of left morphology in one case, and of right morphology in the other two. Further anatomical variation was then found in each group. Nine of the 15 with absence of the left atrioventricular connexion had the right atrium connected to a dominant left ventricle in presence of a rudimentary and incomplete right ventricle associated with discordant ventriculo-arterial connexions, all of them being in usual atrial arrangement and three with pulmonary stenosis. The remaining six in this first variant had the right atrium connected to a dominant right ventricle. In the five patients with imperforate left atrioventricular valves, two had discordant and three had concordant ventriculo-arterial connexions. In the three cases with isomerism, two had absence of the left atrioventricular connexion, with a dominant right ventricle. The last patient had an imperforate left atrioventricular valve and a discordant ventriculo-arterial connexion. From the functional viewpoint, there were 14 patients (10 with absence of an atrioventricular connexion, four with imperforate atrioventricular valve) with congestive heart failure and nine patients (five from the first, one from second, and three from the third variant) with hypoxia. Long-term follow-up (median 16.4 months--varying from 1 to 41 months--in the group with congestion and 27.7 months--varying from 12 to 57 months--in those with hypoxia) showed favorable clinical evolution in 11 (91%). We conclude that an anatomico-functional division can point towards the most appropriate management in this complex anomaly.

Case report: MRI evaluation of congenital coronary artery fistulae.

Congenital coronary artery fistula is a rare disease and MRI is a promising technique that may be useful to demonstrate the coronary artery tree. We report three patients who underwent cardiac MRI to investigate right coronary artery fistulae. On clinical examination, a continuous murmur was heard along the left sternal border, and chest X-ray showed moderate cardiomegaly with enlargement of right chambers in all patients. Transthoracic Doppler echocardiography showed fistulae in two cases; the third case was not demonstrated by transthoracic or transoesophageal echocardiography. MRI demonstrated the course of the fistulous vessels in all patients. All patients underwent surgical closure of their coronary artery fistulae. MRI may show detailed anatomy of congenital coronary artery fistulae and may be useful as an additional non-invasive method in their investigation.

Uhl's anomaly. Differential diagnosis and indication for cardiac transplantation in an infant.

We report the case of an 8-month-old female infant with Uhl's anomaly, who underwent successful cardiac transplantation. The clinical findings, complementary laboratory tests, anatomic findings, and differential diagnosis of the anomaly are discussed.

[Origin of left coronary artery from right and left pulmonary arteries--clinical, anatomico surgical and follow-up evaluation of 3 cases]. / Origem da artéria coronária esquerda das artérias pulmonares, direita e esquerda--avalia ção clínica, anatomocirúrgica e evolutiva de três casos.

Three infants with anomalous origin of left coronary artery, being two from the right pulmonary artery and one from the left, without other congenital defects, ages of 5, 15 and 3 months old, presented with early congestive heart failure due to anterolateral myocardial infarction. Anatomical characteristic was peculiar in two cases since the left coronary artery, before the anastomosis with the right pulmonary artery, presented a wy, 15 mm extension path within the aortic wall which has facilitated the surgical correction by the proximity of these structures. In the third case, the left coronary artery was anastomosed with the left pulmonary artery, 2 cm from its origin and anterior to the left atrium being the surgical correction by direct implant at the ascending aorta. Post-operative follow-up, 36, 30 and 33 months respectively, showed clinical and laboratorial normalization except for the second case in which ST segment remained upward due to ventricular aneurysm. Myocardial recovery, even after extensive infarction, in this anomaly, emphasizes the need for earlier diagnosis and management.

[Pulmonary atresia with intact ventricular septum. Natural development of the right ventricle]. / Atresia pulmonar com septo interventricular integro. Desenvolvimento natural do ventrículo direito.

A case of pulmonary atresia with intact ventricular septum and a hypoplastic right ventricle, with three wall formed portions, that became naturally well developed until 2 years of age, is related. A Blalock-Taussig shunt had been performed early in life. Z value of tricuspid valve of 0.3 and left ventricular in ECG has been changed to a well developed tricuspid annulus of 22 mm diameter and to a right ventricle overload after 2 years of follow-up, being possible surgical correction at that time. This observation, until today unknown, the natural increase of a congenitally hypoplastic cardiac chamber, favors the possibility to postpone the corrective operation to a better stage in this anomaly, when the risk becomes lower.

[Complete atrioventricular septal defect. Anatomo-functional correlation between patients with and without Down's syndrome]. / Defeito total do septo atrioventricular. Correlação anatomofuncional entre pacientes com e sem síndrome de Down.

PURPOSE: To compare clinical course, causes and symptoms beginning enset time in children with complete atrioventricular canal with and without Down's syndrome. METHODS: Records of 80 patients < 2 years of age, were reviewed. There were 55 (69%) with Down's syndrome-group I (GI) and 25 (31%) without-group II (GII). Age at synpton enset intensity, functional class, clinical repercussion and anatomic variations in patients undergoing corrective surgery were evaluated. RESULTS: Mean age at symptoms onset was similar for the two groups (50 +/- 75 days). Class II (NYHA) was more frequent in GI (31 patients-56.5%) and class III-IV (NYHA) in GII (19 patients-76%) p < 0.005. Clinical repercussion evaluation showed that congestive heart failure was present in 34 (62%) patients of GI and 21 (84%) of GII; and, pulmonary hypertension was in 21 (38%) patients of GI and 4 (16%) patients of GII p < 0.04. Mean pulmonary arterial pressure of 50 mmHg or more was present in 68% of children with Down's syndrome and in 35% of GII. Clinical course until surgical correction was down hill in 33 (60%) from GI and 21 (84%) from GII p < 0.03. Seventy seven patients underwent surgical correction. CAVC type A of the Rastelli classification was predominant in both groups, GI 37 (67%)-GII 25 (100%). There or more severe valvar morphologic lesions in group II (38%) than in group I (8%). CONCLUSION: There seems to be a pulmonary vascular hyperreactivity predominance in Down's children and cardiac insufficiency signs in the normal genetic group.

[Transposition of the great arteries with posterior aorta]. / Transposição das grandes artérias com aorta posterior.

Two cases of transposition of the great arteries (TGA) with posterior aorta are reported. The first was submitted to the Senning procedure with good outcome; the other had the diagnosis of double outlet right ventricle with severe pulmonary stenosis, and a Blalock-Taussig shunt was accomplished for hypoxic crisis. The patient died 11 days after surgery and the necropsy demonstrated TGA with posterior aorta. In both cases there was a sub-pulmonary infundibulum. The presence of ventricular septal defects allowed aortic-mitral fibrous continuity, a finding previously described in anatomical reports. Clinical and angiographic aspects are discussed.

[Anomalous origin of the left coronary artery. Concomitant correction with valvar replacement for mitral insufficiency]. / Origem anômala de artéria coronária esquerda. Correção concomitante à substituição valvar por insuficiência mitral.

A four year-old boy with an anomalous origin of the left coronary artery (LCA) directly from the right pulmonary artery was reported. He had been underwent to mitral valve replacement by a St Judes no. 29, due to severe mitral insufficiency, secondary to an extensive myocardial infarction including the anterior and posterior papilary muscles, beside the implantation of the LCA in the aorta. Instead of this favorable outcome, it's emphasized the need for earlier recognition to avoid risk sequelae.

[Ischemic cardiomyopathy in a child. Case report with accentuated coronary disease]. / Miocardiopatia isquêmica na criança. Relato de caso com acentuada coronariopatia.

Ischemic cardiomyopathy in childhood is related with congenital and acquired pathologies which could lead to serious cardiac sequelae as myocardial infarction and sudden death. Unfortunately, when coronary artery malformations are excluded, it is difficult in some cases to be completely certain on the etiology of the arterial coronary disease, due the lack of pathognomonic diagnostic tests. Case report of 6 year old child, with severe coronary artery disease: aneurysm of main branch of left coronary artery; left anterior descending branch and right coronary artery occlusions with accentuated myocardial dysfunction. We believe that the diagnosis of Kawasaki disease is presumptive in this patient. The principal aspects to elucidate the etiology of coronary artery disease are analysed, as well as the importance of identifying patients without known previous illness, but with severe coronary artery lesions, as in this reported case.

Ebstein's anomaly with coarctation of the aorta. An unusual association.

Ebstein's anomaly with coarctation of the aorta is an extremely unusual condition. In this report, the clinical and surgical features of 3 male patients, aged 7 months, 4 years and 14 years, are discussed. All patients were in situs solitus. The first 2 patients had atrioventricular and ventriculoarterial discordance and progressed to heart failure in the neonatal period. The third had atrioventricular and ventriculoarterial concordance, as well as Wolf-Parkinson-White syndrome, with frequent episodes of paroxysmal tachycardia. The 3 patients underwent surgery for correction of the coarctation of the aorta. The patient with atrioventricular and ventriculoarterial concordance underwent tricuspid valvuloplasty using a DeVega-like technique. In addition, ablation of 2 anomalous pathways (Kent bundle), which were detected by the electrophysiologic study, was also subsequently performed. The 3 patients showed a good postoperative outcome for 2 years, although, in those with discordance, the surgical procedure did not influence the dysplasia of the tricuspid valve, because this valve showed light to moderate dysfunction.

[Mitral valve dysfunction caused by papillary muscle necrosis in patients with anomalous origin of the left coronary artery from the pulmonary trunk]. / Disfunção da valva mitral por necrose de músculo papilar em origem anômala de artéria coronária esquerda do tronco pulmonar.

The clinical evolution of mitral valve disfunction caused by papillary muscle necrosis in patients with anomalous origin of the left coronary artery from the pulmonary trunk was analysed in three children (one 42-day-old male, one five-month-old female and one seven-year-old female). Two patients had been submitted to surgical repair of the anomalous origin of the coronary artery; one of them without mitral repair had a bad evolution in the postoperative period, was reoperated for mitral valve replacement and died two days after surgery. The other patient with plastic of mitral valve had a good outcome. The data indicated that the surgical repair of mitral valve should be provided in cases of mitral valve incompetence secondary to anomalous origin of the left coronary artery from the pulmonary trunk.

[Transluminal angioplasty of aortopulmonary anastomosis by the Jatene surgical technique. Report of 2 cases]. / Angioplastia transluminal da anastomose aorto-pulmonar da cirurgia de Jatene. Aplicação em dois casos.

Two patients with transposition of the great arteries submitted to Jatene surgery at ages of 6 days and 6 months respectively, presented in the postoperative period stenosis of the aorto-pulmonary anastomosis. The diagnosis was made three and six years after the surgery. In both patients successful percutaneous transluminal angioplasty (PTA) was performed, respectively six months and immediately after the diagnosis. Pressure gradients decreased from 83 mmHg to 24 mmHg in the first case, and from 76 mmHg to 13 mmHg in the other case, with clear improvement of the angiographic image. Complications of the procedure did not occur in any case. This application for PTA was not previously reported and should be considered as an alternative to the surgical correction of the "new" pulmonary trunk stenosis in the Jatene surgery.

[Treatment using balloon catheterization of pulmonary valve stenosis]. / Tratamento por cateter-balão da estenose pulmonar valvar.

From October 84 to September 88 we performed pulmonary valvuloplasty with balloon catheter (PVBC) in 90 patients (pt) with pulmonary valve stenosis (PVS). The patients were divided in 3 groups (Gr) by their ages: group I (Gr. I) 7 pt (8%) aged 4 days to 11 months, Gr. II 66 pt (73%) aged 1 year to 12 years and Gr. III 17 pt aged 13 to 34 years. This study consists of clinical and echocardiographic evaluation adopting the following parameters of indication: a) transvalvar pulmonary gradient (G); b) absence of dysplasia or hypoplasia of the pulmonary annulus. There were associated lesions which were found in each group: Gr. I atrial septal defect (ASD) in 2 pt and tricuspid insufficiency (TI) in 1 pt, in Gr. II supra valvar stenosis (SVS) in 1 pt and patent arterial ductus (PAD) in 1 pt and in the Gr. III (ASD) in 1 pt. The results after pulmonary valvuloplasty with balloon catheter (PVBC) were: (Gr.I) in 5 pt the G were reduced below 50 mmHg and the average of right ventricular systolic pressure (RVSP) changed from 92 to 62 mmHg and the average of G from 76 to 44 mmHg. One of these pt was submitted to surgery 1 month after the procedure and 2 developed restenosis in 2 and 3 years respectively, being submitted to another dilatation with good results. The 2 pt in which the procedure was not effective were submitted to surgery. In the second group the PVBC was effective in 61 pt (92%) showing the RVSP changed from 118 to 64 mmHg and G changed from 86 to 23 mmHg.(ABSTRACT TRUNCATED AT 250 WORDS)