[Absence of right and left atrioventricular connexion]. / Ausencia de conexión atrioventricular derecha e izquierda.

Fifty seven hearts with absence of atrioventricular (A-V) connection were studied morphologically to specify their types of ventriculoarterial connection and their associated anomalies; the anatomic features of the hearts were correlated with their echocardiographic and cardioangiographic images in order to establish their mutual correspondence. Fifty six hearts had situs solitus; fifty specimens had right absent A-V connection and six had left absent A-V connection. One had situs inversus. All the specimens had: A deep A-V sulcus at the site of the absent A-V connection, a dimple in the muscular floor of the involved atrium connected with the dilated and hypertrophic left ventricle, incomplete right ventricle without inlet portion, ventricular septal defect of variable dimensions (it was obliterated in two), atrial septal defect, the ventricular septum deviated from the crux cordis. The left absent A-V connection had ventricular inversion and discordant ventriculoarterial connection. In the right absent A-V connection the ventriculoarterial connections were concordant in thirty eight hearts, from which thirty four had pulmonary stenosis both infundibular and valvular (five had the tetrad of Fallot), two had pulmonary valve atresia and two had a dilated pulmonary artery; discordant in nine hearts, one with aortic atresia; double outlet, from the right ventricle in two, (one with the tetrad of Fallot) and from the left ventricle in one. The heart in situs inversus had ventricular inversion, right absent A-V connection (left-sided), single (right) ventricle and atresia of the left ventricle. The correlations between cardiac morphology and imaging were precise. Developmentally, this cardiopathy is the result of an ectopic unequally lateralized septation of the common atrioventricular canal, which separates two canals, one stenotic leading to atresia and the other which develops too wide.

[Persistent common trunk. Anatamopathological study of 25 specimen]. / Tronco común persistente. Estudio anatomopatológico de 25 especímenes.

In order to inform the pathologic features and their associated anomalies twenty five hearts with common trunk were studied with the segmental sequential system. An anatomico-embryological correlation was made to understand the pathological complex of this malformation. The results were: type I truncus (96%), infundibular ventricular septal defect (96%), displastic truncal valve (28%), ventriculo infundibular fold (92%), left coronary artery arising from the posterior wall of the truncus (75%), right coronary artery arising from their anterior wall (96%), coronary arteries arising from opposite Valsalva sinuses in the tetracuspid valves; the biventricular conection of the truncus was balanced in 60%, prevailing on the right (16%) or on the left (16%) ventricles. Among the associated anomalies there were right aortic arch, interruption of the aortic arch, anomalous origin of the left subclavian artery, vascular ring, aneurysm of the sinus of Valsalva, and absence of the left branch of the pulmonary artery. Developmentally common trunk is explained as a failure of truncoconal septation in the embryonic heart; a migration arrest of neural crest cells is implicated in the Di George syndrome. Knowledge of the anatomic features of common trunk and their associated anomalies, provides the morphological basis to interpret correctly the clinical diagnostic imagenology.

[Oral enalapril in patients with symptomatic ventricular septal defects]. / Enalapril oral en pacientes con comunicación interventricular sintomáticos.

Experimental and clinical studies have demonstrated that the inhibitors of angiotensin-converting enzyme may reduce the left-to-right shunt over a ventricular septal defect (VSD). The objective of the study is to evaluate the effects of enalapril on short and medium follow-up in patients with symptomatic VSD and Qp/Qs ratio > 1.5 and compare the results with conventional anti-congestive treatment in children. Twenty-four patients with ages from 3 months to 8 years were studied, dividing them in 3 groups of 8 patients each. Group I received treatment with enalapril, group II with furosemide an digoxin, and group III was treated with a combination of the three medicaments. Patients were evaluated after one, three and six months. Evaluation considered clinical signs of congestive failure, weight, heart rate, blood pressure, cardiothoracic index, and with echocardiography the dimensions of the cardiac chambers, size of the VSD and hemodynamic parameters as Qp/Qs, pulmonary artery pressure (PAP), systolic pressure gradient over the VSD, EF and SF. Statistic analysis was done for each group and was compared the three groups. The results demonstrated significant weight gain (p > 0.05) in the three groups. A decrease in heart rate, Qp/Qs and PAP was observed in group III, however without statistic significance. No side effects were observed. The use of enalapril in combination with furosemide and digoxin may have beneficial effects in patients with symptomatic VSD, awaiting surgery.

[The transcatheter closure of patent ductus arteriosus. The initial experience]. / Cierre de conducto arterioso persistente transcateterismo. Experiencia inicial.

Percutaneous closure of the patent ductus arteriosus (PDA) has been recently reported to be an effective alternative in the treatment of patients with ductal shunting. We report the initial experience and results during follow-up of percutaneous ductal occlusion with the Rashkind occluder (USCI) in six patients with isolated PDA. Ages ranged from 3 to 23 years. Diagnosis was corroborated with two dimensional and Doppler echocardiography in all patients. During cardiac catheterization systolic pulmonary artery pressure oscillated between 22 and 64 mmHg and Qp/Qs ratio between 1.3 and 4.1. In two patients prosthesis of 12 mm were used and in the remaining prosthesis of 17 mm. Only one patient demonstrated total occlusion during immediate control aortography, the other patients presented central residual shunting over the occluder. In the three patients occlusion with balloon-catheter was added to the procedure, resulting in total occlusion in two and significant reduction of the shunt magnitude in one. Two technical problems were resolved satisfactorily. None of the cases presented device embolization. Mean follow-up was 23.8 months with control echocardiograms at 24 hours, 1, 4, 12 and 24 months. In all patients immediate reduction of the left atrial dimension was demonstrated. Three patients presented residual shunts in the first 24 hours. In two of them total occlusion had occurred after one month and the other patient persisted with a small residual shunt until one year after the procedure. In conclusion, in this small study group good results were obtained with percutaneous ductal closure.

[Pulmonary balloon valvulotomy. The results in 42 cases]. / Valvulotomía pulmonar con balon. Resultados en 42 casos.

The percutaneous balloon valvulotomy is nowadays the treatment of choice for isolated pulmonary stenosis. Forty two patients with congenital pulmonary stenosis underwent balloon valvulotomy at the Instituto Nacional de Cardiología "Ignacio Chávez". Ages 1 month to 24 years, mean 7.6 +/- 5.9 years, with a follow-up of 28.3 +/- 14.8 months. With an infundibular gradient of 50 mm Hg or more a treatment with propranolol was given. After valvulotomy the total basal pressure gradient decrease from 82.9 +/- 40.6 mm Hg to 31.2 +/- 27.1 mm Hg (p < 0.00001). The patient's results were divided in two groups: with and without reactive infundibular obstruction. The 31 patients without infundibular obstruction had a total initial gradient of 71.6 +/- 33.5 mm Hg, and after valvulotomy the gradient was reduced to 18.9 +/- 11.2 mm Hg (p < 0.00001). The 11 patients with infundibular obstruction had a total initial gradient of 114.8 +/- 43.2 mm Hg, and after valvulotomy the valvular gradient was 12.4 +/- 8.5, the infundibular gradient 53.4 +/- 22.9 and the total gradient 65.9 +/- 29.1 mm Hg (p = 0.002 in relation to the basal gradient). All patients with infundibular obstruction (8 treated with Propranolol) and a follow-up of 8.5 +/- 9.8 months underwent Doppler examination, showing a progressive reduction of the total gradient. By six months no one had a total gradient greater than 50 mm Hg. These data confirm that balloon valvulotomy in valvular pulmonary stenosis is safe and effective, and that restenosis is very low. Good results relay on the use of proper balloon diameters (balloon diameter/pulmonary annulus relation of 1.2 to 1.5).(ABSTRACT TRUNCATED AT 250 WORDS)

[Color doppler-echocardiography study of the triatrial heart]. / Estudio con ecocardiografía Doppler color del corazón triatrial.

Two-dimensional echocardiography has proved its utility in the diagnosis of cor triatriatum sinister by visualizing the defect and its relation to the other structures. Recently pulsed and continuous wave Doppler have been used to determine the degree of obstruction of the membrane. In this study we present three patients with ages six, eight and fourteen months respectively, all with a clinical history of growth retardation, respiratory infections and dyspnea. The patients were studied with two-dimensional and color flow Doppler echocardiography. In all, a membrane was visualized, which divided the left atrium in two chambers, a superior one which received the pulmonary veins and an inferior in continuity with the mitral valve and the left atrial appendage and with color flow Doppler the absence of obstruction at this level was demonstrated. In one patient besides the anomaly of cor triatriatum complete transposition of great arteries with pulmonary stenosis was diagnosed and in another one, patent ductus arteriosus and ventricular septal defect. Diagnosis were confirmed by hemodynamic studies. It is concluded that the color flow Doppler is a diagnostic method of great utility because it permits to obtain more information which complements conventional echocardiography.