Two-dimensional echocardiographic findings in double orifice mitral valve.

Between December 1981 and April 1984, five children ranging in age from 1 month to 5 1/2 years examined by two-dimensional echocardiography appeared to have a double orifice mitral valve. The diagnosis was verified in one patient at surgery, one patient by angiography and one patient by necropsy. Associated malformations included mitral stenosis and regurgitation, coarctation of the aorta, ostium primum and secundum atrial septal defect, ventricular septal defect and hypoplastic left heart syndrome. Three varieties of double orifice mitral valve were observed: an incomplete bridge type (one patient), in which a small strand of tissue connected the anterior and posterior leaflets at the leaflet edge level; a complete bridge type (three patients), in which a fibrous bridge divided the atrioventricular orifice completely into equal or unequal parts and a hole type (one patient), in which an additional orifice with subvalvular apparatus occurred in the posterior commissure of the mitral valve. These three types could be distinguished by sweeping the transducer in cross-sectional view from the apex toward the base of the heart. Both orifices could be seen throughout the scan in the complete bridge type while in the incomplete bridge type the two orifices could be seen only at the level of the papillary muscles. In the hole type, the second orifice was seen at about midleaflet level. In all three types, the chordae surrounding each orifice attached to only one papillary muscle. Congenital mitral stenosis or regurgitation was evident in three patients. The type of the double orifice mitral valve did not predict the presence or severity of symptoms.

Cardiac malformations in trisomy-18: a study of 41 postmortem cases.

The cardiac malformations in 41 karyotyped and autopsy cases of trisomy-18 are presented in detail. The salient findings were a ventricular septal defect in all cases; tricuspid valve anomalies in 33 cases (80%); pulmonary valve anomalies in 30 (70%); aortic valve malformations in 28 (68%); mitral valve anomalies in 27 (66%); polyvalvular disease (that is, malformations of more than one valve) in 38 (93%); a subpulmonary infundibulum (conus) in 40 (98%); a bilateral conus with a short subaortic infundibulum in 1 case with double outlet right ventricle (this being the only documented case of bilateral infundibulum in trisomy-18); double outlet right ventricle in 4 cases (10%), three having a subpulmonary infundibulum only and all 4 having mitral atresia; tetralogy of Fallot in 6 cases (15%), 2 having pulmonary atresia; and a striking absence of transposition of the great arteries and inversion at any level (visceral or cardiac), findings that appear to be characteristic of all trisomies. These data suggest that excessive chromosomal material (as in trisomies) may result in situs solitus at all levels. The malformations of the atrioventricular and semilunar valves were characterized by redundant or thick myxomatous leaflets, long chordae tendineae and hypoplastic or absent papillary muscles. The ventricular septal defect was associated with anterosuperior conal septal malalignment in 25 cases (61%). On the basis of the characteristic valvular lesions, the type of ventricular septal defect and the absence of transposition or inversions, two-dimensional echocardiographic diagnosis of trisomy-18 in the fetus may become possible.

Double-orifice mitral valve: a study of 27 postmortem cases with developmental, diagnostic and surgical considerations.

What is double-orifice mitral valve anatomically and embryologically? In 27 postmortem cases, an anomaly of the tensor apparatus was always found. These malformations may be summarized as: (1) chordal ring; (2) accessory papillary muscle or muscles; (3) subdividing muscular ridge; (4) fused papillary muscles (parachute mitral valve); (5) crossing chordae tendineae; and (6) central fibrous subdivision. More than 1 of these anomalies often coexisted per case. Double-orifice mitral valve almost always consisted of abnormal holes in essentially normal leaflets, rather than of abnormal fibrous bridges or adhesions between normal leaflets. Since these fibrous "bridges" between the smaller accessory orifice and the larger main orifice are composed of mitral leaflet tissue and chordae, not fibrous adhesions, these bridges should not be transected surgically, to avoid iatrogenic mitral regurgitation. The accessory (smaller) orifice was at the anterolateral commissure in 11 cases (41%), at the posteromedial commissure in 12 (44%) and there was a central fibrous subdivision with approximately equal-sized orifices in 4 (15%). The atrioventricular (AV) canal was normally divided in 12 cases (44%) and a common AV canal was present in 15 (56%). When the accessory orifice was at the anterolateral commissure, the AV canal usually was normally divided (8 of 11, 73%). But when the accessory orifice was at the posteromedial commissure, a common AV canal almost always was present (11 of 12, 92%). Functionally, the mitral valve was normal in 13 (48%), regurgitated in 7 (26%) and stenotic in 7 (26%). The key to the diagnostic and surgical understanding of the double-orifice mitral valve is the underlying tensor apparatus.

Wall thickness of ventricular chambers in transposition of the great arteries: surgical implications.

One hundred four specimens of complete transposition of the great arteries (TGA) were studied. The free wall thickness of the left (LV-T) and right (RV-T) ventricular chambers and the left ventricular/right ventricular (LV/RV) thickness ratio for all hearts were analyzed. A control group of 63 normal hearts was also studied. Specimens of TGA were divided into four groups: (1) TGA with intact ventricular septum (TGA + IVS); (2) TGA with ventricular septal defect (TGA + VSD); (3) TGA with a large patent ductus arteriosus (TGA + PDA); (4) TGA with pulmonary stenosis (TGA + PS). In Group I, LV-T was under the 95% confidence limits for normality after 8 months of age, and in Group II, the same was true after 18 months of age. For all groups the RV-T was above the 95% confidence limits for normality from the first month of age. The LV/RV ratio was under 1 in every group. Group I showed a significant decrease in the LV/RV ratio after the neonatal period (p less than 0.005). The potential failure of the left ventricle after anatomic correction of TGA is explained by a multifactorial hypothesis. In view of the ventricular wall thickness findings, we do not recommend the anatomic correction after 8 months of age in Group I or after 18 months in Group II.

[Sudden death and ventricular fibrillation of possible ischemic origin in a boy with hypertrophic myocardiopathy]. / Muerte súbita y fibrilación ventricular de posible origen isquémico en un niño con miocardiopatía hipertrófica.

It has been presented a case of an eleven-year-old patient admitted with a pattern of ventricular fibrillation and diagnosed as hypertrophic cardiomyopathy. Admission analysis and myocardia anatomy evolution suggested ischemic etiology. We checked the risk factors of sudden death, its relation with ischemic disease and the etiology of ischemia in the hypertrophic myocardiopathy.

[Practice Guidelines of the Spanish Society of Cardiology in the postoperative course of congenital heart diseases]. / Guías de práctica clínica de la Sociedad Española de Cardiología en el postoperado de cardiopatía congénita.

Improvements in myocardial protection, surgical techniques, and perioperative care have made it possible to achieve better prognosis in most congenital heart defects. This requires a coordinated, multidisciplinary approach to patient care, based on the preservation of adequate oxygen delivery to vital organs. It is important to have an understanding of normal postoperative status after cardiac surgery so that abnormal postoperative convalescence can be identified and treated.The causes of abnormal convalescence may be grouped into three categories: a) the pathophysiology of the defect before surgery and the acute changes in physiology that result from surgery; b) the effects of hypothermic cardiopulmonary bypass and deep hypothermic circulatory arrest on organ function, and c) the presence of residual anatomic defects. These conditions may result in prolonged convalescence as well as increased morbidity and mortality. Three primary hemodynamic pathophysiologic disturbances may occur during the postoperative period and lead to abnormal convalescence: left ventricular dysfunction, right ventricular dysfunction and pulmonary hypertension. Though sometimes not directly related to either the cardiac defect or surgery, specific problems involving different organs may alter the normal postoperative period. Neurologic, pneumologic, renal, gastrointestinal and infective complications are discussed separately.

[Clinical practice guidelines of the Spanish Society of Cardiology for physical activity in patients with cardiac disease]. / Guías de práctica clínica de la Sociedad Española de Cardiología sobre la actividad física en el cardiópata.

This chapter includes general recommendations regarding the eligibility for recreational and competitive sports participation. Although based on the latest knowledge in sports medicine and cardiology, these recommendations should be updated according to advances in the understanding of the athlete's heart. Physical exercise plays an important role in the leisure time of our society. Many patients with cardiac disease don't want to give up their physical or sport activity programs. Moreover, there is no doubt that many cardiovascular abnormalities may benefit from a controlled physical exercise program. Understanding the cardiac adaptations to exercise training, individualised evaluation of each cardiac pathology and adequate exercise prescription is essential in order to minimize the risks for every cardiac patient.

[Graves' disease in patients with Down syndrome]. / Enfermedad de Graves en pacientes con síndrome de Down.

Three patients showing the rare association of Down syndrome and Graves' disease are reported. While two of the patients were asymptomatic, the third showed goiter, nervousness, weight loss, and tachycardia. In addition to the typical features of hyperthyroidism, this patient showed right heart failure and hypertransaminasemia, which disappeared with antithyroid treatment. Because Graves' disease is rare in children, and the clinical presentation was unusual in one of our patients, we report three patients with Graves' disease and Down syndrome, and emphasize the importance of periodic evaluation of thyroid function in children with Down syndrome not only to detect hypothyroidism.

Social and medical problems in children of heroin-addicted parents. A study of 75 patients.

Between January 1985 and December 1987, seventy-five children of intravenous heroin-addicted parents (one or both) were studied. Their ages ranged from 4 days to 14 years. All patients had suffered from several pediatric diseases. Three major types of problems were found among the children studied: infectious diseases, nutritional diseases, and parental neglect and/or disinterest. The most common diagnoses at discharge were gastroenteritis (24%), pneumonia (21%), malnutrition (17%), upper airway infectious diseases (13%), septicemia (12%), child abuse (4%), acquired immunodeficiency syndrome (3%), and other infectious diseases (24%). Their parents reported hepatitis B virus infection, acquired immunodeficiency syndrome (AIDS), and alcoholism. The unemployment rate among the fathers was 37%. Sixteen percent of mothers were prostitutes. There was an imprisonment record of 19% for mothers and fathers combined. A multidisciplinary approach for this group of children would make prevention possible and care less expensive.

[Atrial septal defect symptomatic in infancy. Report of 11 cases (author's transl)]. / Comunicación interauricular mal tolerada en el lactante. Estudio de 11 casos.

Eleven cases of atrial septal defect, ostium secundum, with important symptomatology, under the age of one year are reported. The clinical picture does not differ essentially from what is found in older patients. Extracardiac associated anomalies are frequent. Pulmonary to systemic flow ratio varied between 1.3 and 3.5. The E.C.G. showed typical findings is most of the cases, but unexpected left ventricular enlargement was found in one case, and in another one with a Noonan syndrome, a left anterior hemiblock was present. Surgery was performed in six patients, between one and six years. In all, direct vision of the defect showed the large size of it. Pulmonary vascular resistances were normal. It is postulated that early vasodilatation of the pulmonary vascular bed could be the origin of the early onset of a left to right shunt in this patients without an increase in pulmonary vascular resistances.

[Children of heroin-addicted parents: a risk group. Study of 110 cases]. / Hijos de padres heroinómanos: un grupo de riesgo. Estudio de 119 casos.

In order to know the medical and social problems of children of heroin addicted parents (one or both), one-hundred and nineteen patients were studied. All children had been hospitalized suffering from different diseases. The age of these children ranged from 1 day to 17.3 years (19.6 +/- 30.9 months). Seventy-four of the children were less than 1 year old. The total number of admissions was 159 (range 1-14), representing 2.575 days of hospitalization. The most frequent diagnoses were infectious diseases (gastrointestinal and respiratory), deficiency related diseases (malnutrition and anemia) and parental neglect. Maltreatment was suspected in 44 children (physical: 10, severely neglected: 26, abandonment: 8). Eight children died during the study (AIDS: 4, maltreatment: 2, other causes: 2). Nine parents also died (4 from heroin overdose). The social and family environment of these children, with an increased risk for delinquency and marginality, as well as frequent hospitalizations, make these children a special group at risk for social and health problems which demands multidisciplinary attention and understanding.

[Diagnostic sequence for congenital cardiopathies]. / La secuencia diagnóstica de las cardiopatías congénitas.

Nomenclature for congenital heart malformations has always been confusing. Efforts of classifying these malformations according to the relations and morphology of the cardiac segments have been attempted but have risen confusion because there many terms for the same malformations. In this paper we describe a well defined sequencial diagnosis that links the cardiac segments, and besides, permits the identification of the associated defects. The connections between the cardiac segments are considered has the most important step in the classification of heart malformations while, relations are left as a secondary stage in this study. In some congenital hearts malformations concordant or discordant atrioventricular connections do not apply as the univentricular hearts or ambiguus atrial situs, but we also stablish the parameters for their classification. In our opinion, this nomenclature describe in a complete and objective way the congenital heart malformation.

[Ventricular septal defect in the first year of life (author's transl)]. / Comunicación interventricular en el primer año de la vida.

Thirty cases of CIV in the first year of life are presented. Association with prematurity and other malformations anticipate cardiac failure. Left ventricular hypertrophy is more frequent in conal defects. Repolarization disturbances were found in 73% of the cases in left precordial derivation with significative relief after surgical repair (banding). Conduction disturbances after surgical closure of ventricular septal defect was found in 15% of the cases. Surgical treatment during the first year was needed in 86% of patients. Only one case had good evolution without surgery. After four months of life total correction of septal defect as prefered. In patients with previous pulmonary artery banding, correction must be made one or two years later.