[Changes in oxygenation indexes with the use of nitric oxide in the postoperative period of congenital heart disease surgery in patients with severe pulmonary hypertension]. / Cambios en los índices de oxigenación con el uso de óxido nítrico en el postoperatorio de corrección de cardiopatías congénitas con hipertensión pulmonar severa.

UNLABELLED: In this clinical study, 7 pediatric patients with severe pulmonary artery hypertension, secondary to congenital heart disease, received inhaled nitric oxide at doses of 20 ppm in the acute postoperative management of congenital heart repair for 3.5 days. Monitoring included oxygenation index, alveoloarterial oxygen difference, pulmonary and systemic pressure, measurements were recorded previous to the NO administration; 1, 6, 12, 24, 36, 48 and 72 hours after treatment with NO, nitrous oxide (NO2) and seric methemoglobin also were monitored. RESULTS: Average age was 3.6 years. The oxygenation index before inhaling NO was 166 +/- 100, 72 hours following inhalation of NO the oxygenation index was 210 +/- 98. The alveolo-arterial baseline oxygen difference was 270 +/- 145; 72 hours after inhaling NO, it was 163 +/- 167. No decreases in pulmonary and systemic arterial pressure during NO inhalation occured. The levels of NO2 and seric methemoglobin were not toxic. CONCLUSIONS: Treatment with inhaled NO after cardiac surgery in children with severe pulmonary artery hypertension secondary to congenital heart disease may be useful to improve oxygenation with no changes in pulmonary and systemic arterial pressure. Inhaled NO at 20 ppm did not cause toxicity.

[One-and-a-half correction. Results at the National Institute of Cardiology "Ignacio Chávez"]. / Corrección uno y medio. Resultados en el Instituto Nacional de Cardiología "Ignacio Chávez".

Herein we describe a preliminary report of 4 patients with hypoplastic right ventricle and 3 patients with Ebstein's anomaly who undergone "one and a half" repair at the Instituto Nacional de Cardiología "Ignacio Chávez". There was not surgical mortality and at mid-term follow up the patients are doing well.

[Electrocardiography and echocardiography aspects of hypertrophic myocardiopathy in pediatrics]. / Aspectos electrocardiográficos y ecocardiográficos de la miocardiopatía hipertrófica en edad pediátrica.

Hypertrophic cardiomyopathy (HCM) is a heterogeneous disease, characterized by asymmetric hypertrophy of the left and/or right ventricle with disarray of myocardial fibers. In order to know its clinical and electrocardiographic manifestation in the pediatric age group, we made a retrospective study of 24 cases from 1986 to 1995. There were: 15 girls and 9 boys, with a mean age of 6 years (age range: 1 month to 17 years). Clinical manifestations were dyspnea (71%), syncope (42%) and palpitations (42%). Physical examination disclosed an aortic systolic murmur in all patients, a mitral regurgitation in 42% and physical signs of congestive heart failure in 54% of patients. Chest X rays showed cardiac enlargement in 71% and pulmonary capillary hypertension in 42%. The most frequent ECG abnormalities were: a prolonged time in the intrinsecoid deflection onset on leads corresponding to the affected region, more or less deep and clean Q waves on leads aVF, aVL, V5 and V6, as well as supraventricular and ventricular rhythm disturbances in 11 patients (46%) with and without congestive heart failure. Bidimensional echocardiography confirmed antero-septal hypertrophy in all patients. The mortality rate was 17%. HCM is rare disease in the pediatric age group. Mortality increases when congestive heart failure and arrhythmias are present. Treatment must be individualized in all cases.

[Chromosome 22 (22q.11.2) deletion. Etiology of conotruncal heart abnormalities]. / Deleción en el cromosoma 22 (22q.11.2). Etiología de cardiopatías congénitas troncoconales.

Important advances in the diagnosis and treatment of congenital heart disease (CHD) have been made in the past 50 years. Nowadays echocardiogram plays an important role in the diagnosis. This procedure is able to identify a wide range of malformations. Cardiac catheterization is mainly a therapeutic tool, surgery is now performed much earlier because CHDS are diagnosed sometimes before birth or very early in life. All this advances in the diagnosis and treatment of this group of patients, allows them not only a better quality of life but also the possibility of reaching adulthood and having children. The study of the etiology of CHD is a field that has not evolved as fast as the assessment and treatment. Nowadays we have a larger population of adults with CHD. The discovery of a microdeletion of chromosome 22 q11.2 associated with conotruncal cardiac defects, proves a common etiology for clinical phenotypes and conotruncal malformations. In order to identify, which of these patients share the same etiology and presented with this syndrome, we collected a group that shared not only heart defects of the conotruncal type but also specific phenotypic alterations such as broad nasal bridge, nasal dimple, high palate, and digitalization of the first finger among others. The first two patients studied with FISH technique were positive to monosomy of a locus on chromosome 22. Those patients with CHD of conotruncal type should undergo microdeletion testing so genetic counseling can be offered as well as appropriate treatment in areas such as cardiology and developmental psychology.

[Fenestrated Fontan surgery in high risk patients]. / Cirugía de fontan fenestrado en pacientes de alto riesgo.

A retrospective analysis is presented of all patients who had fenestrated Fontan procedure between january 1990 and may 1996. Surgery was indicated in the presence of anyone of the following risk factors: mean pulmonary pressure higher than 20 mmHg; pulmonary vascular resistance higher than 2 UW; ejection fraction less than 60%; systemic ventricular end diastolic pressure higher than 8 mmHg; Nakata index less than 200 mm2/m2, McGoon index less than 2. The diagnosis were: Absent right atrio-ventricular connection with concordance ventriculo-arterial connection 10 patients; pulmonary atresia with intact septum, 1 patient; Ebstein's malformation, 1 patient and absent left A-V connection with discordance VA connection, 1 patient. The mean of age was 6.7 years (range 2.5-11 years). Overall mortality was 23%. No significant difference in risk factors was found between survivals and no survivals. Nonsurvivors had between two an four risks factors. Postoperative complications were 1 patient with protein losing enteropathy and stroke (1 patient). The mean duration of pleural effusion was 16 days (range 4-45 days). We consider fenestrated Fontan procedure useful for patients with congenital heart disease with a one hypoplastic ventricle and one o more risks factors.

[Fontan surgery in tricuspid atresia. Experience at the National Institute of Cardiology "Ignacio Chávez"]. / Cirugía de Fontan en atresia tricuspídea. Experiencia del Instituto Nacional de Cardiología "Ignacio Chávez".

A retrospective analysis is presented of all patients under 18 years of age with tricuspid atresia and concordant ventriculo-arterial connection who underwent a Fontan procedure in the National Cardiology Institute "Ignacio Chávez", México-City, between January 1989 and December 1995. In this period Fontan procedure was performed in 22 patients with a mean age of 6.52 +/- 1.4 years. 14 of them were females. Thirteen patients had previous palliative procedure. Age of palliation was 10 +/- 12 months with period of 5 +/- 2 years between palliative and Fontan procedure. Overall mortality was 31% (7 patients); with a mortality of 42% between 1989 and 1992, of 22% between 1992 and 1995 and of 17% since 1994 with all 5 patients operated in 1995 surviving. Mean pulmonary pressure was 13.9 +/- 4.15 mmHg, mean pulmonary vascular resistance 1.66 +/- 0.57 U.W/m2, left ventricular end diastolic pressure 7.5 +/- 2.46 mmHg, ejection fraction 58 +/- 11% and Nakata and McGoon indexes 219 +/- 56 mm2/m2 and 2 +/- 0.1 respectively. No significant differences were found survivors and non-survivors, with exception of the left ventricular end-diastolic pressure (p < 0.05). Causes of death were multiple organ failure in 2, ventricular fibrillation in 3 and septic shock in 2.

[Bidirectional cavopulmonary diversion for tricuspid atresia. Experience in the National Institute of Cardiology]. / Derivación cavopulmonar bidireccional para la atresia tricuspídea. Experiencia en el Instituto Nacional de Cardiología.

Bidirectional cavopulmonary shunt is an alternative palliative procedure for patients with congenital cyanotic heart disease, specially those patients less than "ideal" candidates for a Fontan's procedure. We present our results with this shunt in patients with tricuspid atresia. Twenty patients with tricuspid atresia were operated on with this shunt, with these associated defects: 20 atrial septal defect, 17 ventricular septal defect, 10 pulmonary stenosis, 1 pulmonary atresia and 1 transposition of the great arteries. Sex: 10 males and 10 females; the age was 27 days to 6 years (mean 1.8 years), the weight was 3.2 kg to 24 kg (mean 10.7 kg), the mean pulmonary artery pressure was 11 to 24 mmHg (mean 17 mmHg), pulmonary vascular resistance was 1.5 to 5 UW (mean 3.1 UW). Postoperative oxygen saturation improved 15 to 120%. All patients survived the surgical procedure. Three patients died in the immediate postoperative period, 2 due to a complications in the postoperative period and 1 due to sepsis. There were two late deaths, 1 sudden death after 6 months of the shunt, and 1 due to sepsis after a Fontan's procedure. Four patients presented pleural effusion and 2 pericardial effusion, they resolved well. We have 15 patients alive and well, in functional class I, and minimal cyanosis. We can conclude that this surgical procedure is useful in the management of patients with tricuspid atresia.

[Interatrial aneurysm as a cause of supraventricular arrhythmia in a newborn infant]. / Aneurisma interatrial como causa de arritmias supraventriculares en el neonato.

Atrial septal aneurysm is a rare malformation that may occur as an isolated abnormality or in association with various cardiac defects or with connective tissue disease. The reported incidence in adults is 0.2% and in children 4.9% which suggests spontaneous regression with advancing age. Atrial septal aneurysm may be complicated by thromboembolism, valvular obstruction or arrhythmias. Supraventricular arrhythmias have been reported in as much as 16% of the cases, most commonly paroxysmal supraventricular tachycardia, supraventricular extrasystoles and atrial flutter. The relationship between the arrhythmias and the atrial septal aneurysm is still controversial. Two cases of atrial septal aneurysm in neonates are described, who presented with arrhythmias: supraventricular extrasystoles with aberrant conduction and atrial flutter with variable conduction, respectively. One of them required treatment with various anti-arrhythmia agents. During follow-up the arrhythmias remitted with spontaneous involution of the aneurysm. In the presence of the supraventricular arrhythmias in neonates, atrial septal aneurysm needs to be ruled out as a potential cause.

[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)

[The aortopulmonary window. A report of 4 cases]. / Ventana aortopulmonar. Reporte de 4 casos.

The aortopulmonary window is a rare congenital disease in which the aorta and pulmonary arteries are communicated by a defect of variable diameter. We review the clinical picture, diagnosis, associated lesions and treatment of four patients with this disease in a period between 1980 and 1990. Two patients had a distal and two a proximal type. There were associated lesions in three patients: Aortic arch interruption type A, patent ductus arteriosus, subaortic stenosis and a ventricular defect. All patients underwent surgical correction of a communication between 12 and 15 mm of diameter. We discuss diagnostic problems and treatment of this congenital disease.

[Primary pulmonary arterial hypertension in children under 2 years of age]. / Hipertensión arterial pulmonar primaria en niños menores de 2 años.

A retrospective analysis of 18 patients under two years of age who suffered from primary pulmonary hypertension was made between july 1982 november 1988 in order to define their clinical course and prognosis. Perinatal history was irrelevant. In 13 patients diagnosis was established before the first year of life and in 8 of them in the neonatal period. All patients presented the same symptomatology: Cyanosis, dyspnea and/or repeated respiratory infections. The electrocardiogram showed various degrees of enlargement of the right atrium and right ventricle as well as an uneven ST segment in the precordial leads; these could not be related to the systolic or diastolic pressures of the right ventricle. In the chest roentgenogram the most important and constant feature was the heart enlargement. Cardiac catheterization showed a mean pulmonary systolic arterial pressure of 64.46 mmHg and a mean pulmonary arterial resistance of 6.4 U/m.2 These parameters could not be related to a bad prognosis. Three patients were given vasodilators (hydralazine) with positive clinical and echocardiographic results. Five patients died (27%) two months after the diagnosis was made and three months after the onset of symptomatology, four of them died due to congestive heart failure and one had sudden death. This study shows the early onset of the disease as well as its short term high mortality.

[Rheumatic cardiopathy in children younger than 6 years of age]. / Cardiopatía reumática en niños menores de 6 años.

UNLABELLED: Most of the published papers on Rheumatic Fever (RF) have not included the younger population. We selected 211 cases of children with RF younger than 6 years of age from 9,471 clinical files from 1944 to 1982. These were followed retrospectively to identify the presence of rheumatic activity, subsequent attacks and penicillin profilaxis. From de 211 cases, 209 had carditis; 57% of them were girls and 43% boys. There were no previous infections of the upper respiratory tract in 36% of the patients. The number of cases with RF increased abruptly after 3 years of age and continued increasing until 5 years of age when 70.5% of the population had there first clinically recognized attack. Lesions were present in the mitral valve in 80% of the cases, in the aortic valve in 12%, in the tricuspid in 5% and in the pulmonary valve in 3%. The death rate during the first attack was 20% being refractory heart failure the main cause of death. Thirteen cases suffered rheumatic pneumonia, 9 of whom died (69.2%). CONCLUSIONS: 1) The incidence of acute rheumatic fever in children under 6 years of age has decreased with time. 2) The death rate as well as the valvular damage decreased with the parents cooperation with the treatment. 3) The changes in the clinical picture and the severity of valve sequelea may be due to penicillin profilaxis and the better understanding of the disease.

[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.

[Tetralogy of Fallot with abnormal coronary arteries]. / Tetralogía de Fallot asociada a anomalias de las arterias coronarias.

Between 1980 and 1989 nine patients with Tetralogy of Fallot with abnormal coronary artery underwent surgical treatment. In all but one, the abnormal coronary artery was identified at surgery. Seven (group I) had the anterior descending coronary artery arising from the right coronary artery; one (group II) with had the right coronary artery arising from the left coronary artery and finally, the only case of the group III had left coronary artery arising from the main pulmonary artery. Systemic-pulmonary anastomosis was done in two cases of the Group I, in one due to severe hypoplasia of the pulmonary ring and main pulmonary artery and in the other due to the anomaly of the coronary artery. In the case of the Group II the right coronary artery was accidentally cut and the patient developed right ventricular infarction and died. All surviving are in NYHA functional class I. Nowadays, the preoperative evaluation of Tetralogy of Fallot, can be done by echocardiography despite the possibility of its association with abnormal coronary artery. In special cases aortography or arterial coronarography should be considered.