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The authors present three cases of pregnant women with symptomatic severe mitral stenosis with a mean age of 28.6 +/- 2.3 years, and during 27.6 +/- 1.52 weeks of pregnancy. Two patients were in class III and one in class IV of the New York Heart Association (NYHA). All patients had a mitral valvular area equal or less than 1 cm2, with a Wilkins score of 7 to 9 and mitral insufficiency grade I in two cases; two, had severe pulmonary arterial hypertension (mean > 50 mm Hg). After Percutaneous Mitral Valvuloplasty (PMV) the mitral valve measured by 2D echocardiography increased form 0.83 +/- 0.2 cm2 to 1.8 +/- 0.15 cm2; the mean transmitral gradient diminished from 13 +/- 3.4 mm Hg to 3.6 +/- 1.15 mm Hg; the degree of mitral insufficiency was no modified in neither case. Hemodynamic results revealed increasing of the mitral valve from 0.83 +/- 0.18 cm2 to 2.23 +/- 0.3 cm2; the mean mitral gradient decreased from 21.6 +/- 9 to 4.3 +/- 0.5 mm Hg; the mean left atrial pressure from 30 +/- 12 to 12.3 +/- 4 mm Hg; the mean pressure of the pulmonary artery diminished suddenly from 44.3 +/- 16 to 25.6 +/- 11 mm Hg. The average fluoroscopic time was 15.3 +/- 3 minutes. There were no complications. The patients were discharged 48 hours after the procedure and continued their pregnancies in class I NYHA, which resolved in a non complicated vaginal delivery with normal products. We conclude that PMV is a safe and useful therapy in pregnant patient with severe mitral stenosis refractory to medical treatment.

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The authors describe the morphogenesis and functional alterations of the coronary arterial net in the ectopic coronary arteries: a) with origin in the aorta or its branches and b) with origin in the pulmonary artery. The coronary arteries are developed from: 1) endothelial sprouts localized in the great arteries walls at the level of the sigmoidal values, 2) right and left subepicardial vascular network and 3) the intramyocardial sinusoids. Most of the ectopic coronary arteries result from alterations in the connection between these three embryonic elements. The deviation of one of the subepicardial vascular network in a wrong way (in direction of pulmonary artery or the opposite Valsalva sinus) will stimulate the development of endothelial sprouts which will connect such network originating abnormal connections and anomalous origin of the coronary arteries. The origin of both coronary arteries from the pulmonary artery is in compatible with life. Myocardial ischemia is absent in patients with type I (infant) or type II (adult) anomalous origin of one coronary artery from the pulmonary artery, only in the transitional phase between both types (I and II) there is myocardial ischemia previous to the formation of the collateral coronary circulation. The ectopic origin of the coronary artery from the aortic Valsalva sinus have very little hemodynamic repercussion in the patient. Although there are cases with postexercise sudden dead. These anomalies associated to atherosclerotic coronary stenosis have an impact on the evolution and prognosis of ischemic heart disease.

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In order to explain the congenital coronary arteries malformations, the authors review the recent concepts on the coronary artery morphogenesis, based in the findings that in the human embryo, these arteries evolve from three sources: 1) endothelial aortic buds, 2) cavitary cellular groups from pericardial origin and with angiogenic character, which migrate to the cardiac zones where the coronary arteries will be distributed, and 3) the intramyocardial sinusoids. The anatomic and histologic cardiac alterations will be reflected in modifications of the coronary artery pattern. The coronary artery fistulae are formed by the persistence of the sponge structure of the myocardial wall, present in the early ontogenic steps of the cardiac development; such fistulae alter the normal functions of the coronary vascular tree and are capable to cause angina pectoris to the patient through diverse mechanisms: absence of capillarization, steal phenomenon aggravated by the altered coronary arteries properties when aneurysm or vascular channels are developed. The authors suggest a classification of the congenital coronary arteries anomalies: I. Anomalous origin in the sinus of Valsalva (anomalous and ectopic origin), II. Malformations of the coronary branches (in number, distribution and wall anomalies) and III. Anomalous connection of the coronary arteries: fistulae and persistence of the intramyocardial sinusoids isolated or communicated to left and right ventricles. The latter are frequently associated with aortic or pulmonary valve atresia. They do not cause myocardial ischemia and are formed secondary to the intracavitary elevated pressure which maintained the persistence, dilatation and communication of the ventricular chambers with such sinusoids and coronary arteries in the case of pulmonary valve atresia and with coronary veins in the case of aortic valve atresia.

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The congenital absence of the pulmonary valves is a rare malformation that mainly affects children, frequently is associated with other cardiac malformations and very rarely is present in adults. Elective or urgent surgery is indicated in the symptomatic child; on the other hand, surgical treatment in the asymptomatic adult with this anomaly is controversial. Based on the reported experience in the literature, in the present article, the authors discuss the therapeutic approach of an asymptomatic 22-year-old male with pulmonary valvular absence with practically no hemodynamic repercussion. Although there had been few reported cases of isolated pulmonary valvular absence, the authors concluded that surgery is not a satisfactory solution and that it might be postponed to the time that patients develop congestive heart failure and or respiratory insufficiency. In order to detect that on time, we recommend a follow up based on periodic echocardiographic studies.

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Forty patients with a diagnosis of acute myocardial infarction (anterior 24, and inferior 16) were studied. Of these patients, 37.5% manifested second and third degree atrioventricular (AV) block as a complication; another 30% showed complete right bundle branch and left anterior hemiblock. Right bundle branch and left posterior hemiblock were evidenced in 12.5% of the subjects. There was 20% with complete left bundle branch block. Electrophysiologic studies were performed in all patients to assess the site of block. A direct relation was found between the surface ECG and the His bundle electrogram studies in patients with an inferior myocardial infarction and AV block, both procedures located the conduction disturbances at the AV node (suprahisian block), in contrast to patients with anteroseptal myocardial infarction whose surface ECG only showed bundle branch block or fascicular block. The His bundle electrogram registered multiple levels of AV block, 70% with troncular and infrahisian block that gave way to sudden AV block. The mechanism responsible for this block was considered to be a functional longitudinal dissociation of conduction system due to an acute ischemic injury of the His bundle, more than a sudden and simultaneous failure of all the bundle branch of His. We conclude that electrophysiologic studies are a useful procedure for identification of a group of patients with multiple AV conduction disturbances that have a less favorable prognosis than those with only suprahisian level of block.

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The authors present the clinical cases of 5 patients, with rupture of the aortic Valsalva's sinus aneurysm open to the right chambers, diagnosed clinically, echocardiographically and hemodynamically in the past eight months. Among them, four were males, and one female. Three were treated surgically successfully, one patient refused surgery and another had a sudden death before surgery. The patients showed arteriovenous blood flow shunt, three from the aorta to the right ventricle, and two to the right atrium. One case was associated to an atrial septal defect, and tricuspid insufficiency in another. The authors added a sixth case: a woman, with rupture of the aortic Valsalva's sinus open to the right ventricle, aortic insufficiency and diaphragmatic subaortic stenosis. The authors discuss the embryologic origin of the formation and rupture of the Valsalva's sinus aneurysm as well as the factors that contribute to it's rupture, the natural history of the illness, it's classification, association with other heart disease as well as diagnosis and treatment.

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The most severe arteritis due to Takayasu's disease are those related to renal and coronary arteries. The first one because it produces severe arterial hypertension and the second one because it puts the patient in high risk of suffering either myocardial ischemia or infarction. These situations worsen when this entity is associated to valvular heart lesions. The authors present the clinical cases of two female patients with Takayasu's disease. One of them in acute phase of the illness, where coronary arteritis, mild coarctation of the aorta, right pulmonary artery stenosis, and pulmonary valve stenosis were present. The second patient was seen during the remission phase of the disease with obstruction of the left subclavicular artery, renal arteritis, severe arterial hypertension and aortic valve insufficiency. The authors discuss the prognosis of patients with Takayasu's disease associated to valvular heart disease and its role in the etiology of pulmonary valvular stenosis. Finally, the authors point out the importance of recognizing the active and non active phases of the Takayasu's disease in relation of the adequate stage for surgical treatment of the lesions caused by this disease.

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With the purpose of studying the right ventricular infundibulum response to the obstruction of the pulmonary blood flow, the authors inform the results of the right ventricular volumes, and function changes before and after pulmonary intraluminal valvuloplasty performed in six adult patients with congenital stenosis of the pulmonary valve. After the valvuloplasty, all right ventricular volumes increased but only slightly, except for the end systolic volume at the right infundibulum, which decreased after valvuloplasty (for alpha = 0.10, p less than 0.10). The ejection fraction of this infundibulum increased after valvuloplasty (for alpha = 0.05 p less than 0.03), while the ejection fraction of the inflow chamber remained unaltered. In the same way decreased the work and power of the inflow tract of the right ventricle, regardless the decreased in the ventricular overload post-valvuloplasty; however, the ratio work vs. end diastolic volume of the right ventricle decreased (for alpha = 0.05, p less than 0.03). The authors discuss these results in relation with the changes produced by the obstruction, acute or chronic, of the pulmonary blood flow on the infundibular wall tension and contractility, whose structure and behavior allow to propose that the function of the infundibulum by means of contraction protects the pulmonary vasculature, against right ventricle hypertension.

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The authors present the clinical case of a 31 year female with systemic vascular hypertension secondary to a 65 percent unilateral fibromuscular renal artery stenosis. The centellographic study demonstrated hypoperfusion on the right kidney and serious functional damage; the patient was subject to intraluminal renal angioplasty resulting in right renal arterial thrombosis, which was treated successfully with intra-arterial and intravenous streptokinase infusion. Following the thrombotic therapy, recanalization of the thrombosed artery and normal renal excretion of the contrast substance was observed.

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The spontaneous aneurysm of the ductus arteriosus is an extremely rare disease. From a total of 24 adult cases reported in the literature up to 1987, only in one case the ductus was open and other, was recanalized after being closed. We described the second case in the literature in a patient with totally permeable ductus arteriosus who developed a spontaneous aneurysm and endarteritis, showing the great importance of the diagnostic procedures such as echocardiogram, tomography and angiogram, as well as the surgical treatment which was successful in our patient. The probable etiology of this disease is discussed.

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The authors described the clinical, echocardiographic, and hemodynamic studies, as well as the surgical findings in four adult patients with total anomalous connexion of the pulmonary veins (TACPV); two of them with pulmonary arterial hypertension and T A C P V draining in the coronary sinus, and the other two cases with normal pulmonary artery pressure and T A C P V draining in the superior vena cava. None of the patients had symptoms nor signs of congestive heart failure. All cases had successful surgical interventions. Patients survival into adulthood is discussed.

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We describe the first case of BAS in our country in a three months old child with transposition of the great arteries, restrictive atrial septal defect (RASD) and intact interventricular septum. When he was 15 days old, we performed a balloon atrial septostomy. He had temporal improvement and six weeks later his cyanosis increased, and a new catheterization showed systemic arterial oxygen saturation of 30%, RASD and an interatrial pressure gradient of 2.1 mmHg (left atrium LA: 3.9 and right atrium RA: 1.8). We decided to perform a new septostomy with Park's blade atrial septostomy catheter. After the procedure the interatrial pressure gradient decreased to 0.2 mmHg (RA: 4.3 and LA: 4.5), the angiography shunt and atrial pressures increased. Five months later the child is alive and the systemic arterial oxygen saturation is 51.3%. The technique, advantages and complications are described.

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We describe the first case of balloon pulmonary valvuloplasty performed in this country. This 12 year old girl had a transvalvular gradient of 94 mmHg and after the procedure it improved to 20 mmHg. Four months later, cardiac catheterization did not show any significative change in pulmonary valvular gradient (26 mmHg). We describe the technique, hemodynamic findings, complications, and advantages of this procedure. We also review the literature. We consider that balloon valvuloplasty should be the first choice procedure in the treatment of patients with congenital pulmonary valve stenosis.

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Review is made of the literature of coronary arteriovenous fistula, since its first description up to 1975; we mention the most important points treated by each author. Two cases are studied clinically, by entheterization and coronariography. We take into consideration the differential diagnosis with patent ductus arteriosus, ventricular septal defect with aortic insufficiency, and rupture of congenital aneurysm sinus of valsalva to right cavities. The result of these studies is that, if an atypical continous murmur for an arterial duct is present, we may suspect a coronary fistula as a first possibility, since an aneurysm of valsalva's sinus is a rare entity.

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