1/8. Ebstein's anomaly: one and a half ventricular repair.patients with Ebstein's anomaly can present after childhood or adolescence with cyanosis, arrhythmias, severe right ventricular dysfunction and frequently with left ventricular dysfunction secondary to the prolonged cyanosis and to the right ventricular interference. At this point conventional repair is accompanied by elevated mortality and morbidity and poor functional results. We report our experience with three patients (8, 16 and 35 years of age) with Ebstein's anomaly, very dilated right atrium, severe tricuspid valve regurgitation (4/4), bi-directional shunt through an atrial septal defect and reduced left ventricular function (mean ejection fraction = 58%, mean shortening fraction = 25%). All underwent one and a half ventricular repair consisting of closure of the atrial septal defect, tricuspid repair with reduction of the atrialised portion of the right ventricle and end-to-side anastomosis of the superior vena cava to the right pulmonary artery. All patients survived, with a mean follow-up of 33 months. In all there was complete regression of the cyanosis and of the signs of heart failure. Postoperative echocardiography showed reduced degree of tricuspid regurgitation (2/4) and improvement of the left ventricular function (mean ejection fraction = 77%, mean shortening fraction = 40%). In patients with Ebstein's anomaly referred late for surgery with severely compromised right ventricular function or even with reduced biventricular function, the presence of a relatively hypoplastic and/or malfunctioning right ventricular chamber inadequate to sustain the entire systemic venous return but capable of managing part of the systemic venous return, permits a one and a half ventricular repair with good functional results.- - - - - - - - - - ranking = 1keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
2/8. Transient platypnea-orthodeoxia-like syndrome induced by propafenone overdose in a young woman with Ebstein's anomaly.In this report we describe the case of a 37-year-old white woman with Ebstein's anomaly, who developed a rare syndrome called platypnea-orthodeoxia, characterized by massive right-to-left interatrial shunting with transient profound hypoxia and cyanosis. This shunt of blood via a patent foramen ovale occurred in the presence of a normal pulmonary artery pressure, and was probably precipitated by a propafenone overdose. This drug caused biventricular dysfunction, due to its negative inotropic effect, and hypotension, due to its peripheral vasodilatory effect. These effects gave rise to an increase in the right atrial pressure and a decrease in the left one with a consequent stretching of the foramen ovale and the creation of massive right-to-left shunting. In our case this interatrial shunt was very accurately detected at bubble contrast echocardiography.- - - - - - - - - - ranking = 0.5keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
3/8. Right ventricular diastolic dysfunction and patent foramen ovale causing profound cyanosis.A 73 year old woman presented with profound central cyanosis and a history of a minor stroke. She had normal heart morphology, normal pulmonary artery pressure, and a normal coronary angiography. A patent foramen ovale (PFO) with a massive right to left shunt was demonstrated at the atrial level, with normal pulmonary venous saturations and PO2 values. This rare, age related case of right ventricular diastolic dysfunction in a normotensive patient revealed a generous PFO allowing a pronounced right to left shunt.- - - - - - - - - - ranking = 0.0056673553287647keywords = dysfunction (Clic here for more details about this article) |
4/8. Surgical treatment for isolated hypoplasia of the right ventricle.Four patients with isolated hypoplasia of the right ventricle who underwent intracardiac repair are described. An atrial septal defect (ASD) was successfully closed in 3 patients with mild hypoplasia of the right ventricle. The remaining patients underwent ASD closure and right atrium-to-pulmonary artery connection with a conduit with a bovine pericardial valve because of severe hypoplasia of the right ventricle and tricuspid valve. Postoperative angiocardiogram in the last patient demonstrated two-channel pathways from the right atrium to the pulmonary artery via both the right ventricle and external conduit. At long-term follow-up studies, 3 patients had developed arrhythmias and/or sinus node dysfunction, and one had died, probably because of arrhythmia, 13 years postoperatively. The right ventricular ejection fractions were below normal levels at 11 and 12 years postoperatively in 2 patients who underwent closure of ASD. It was concluded that the mild form of hypoplasia of the right ventricle should be surgically corrected by ASD closure, while right atrium-to-pulmonary artery connection or Fontan's operation should be chosen for cases of severe hypoplasia. Arrhythmias and hypofunction of the right ventricle are long-term problems after intracardiac repair of this anomaly.- - - - - - - - - - ranking = 0.0011334710657529keywords = dysfunction (Clic here for more details about this article) |
5/8. chloramphenicol toxicity associated with severe cardiac dysfunction.A 9-month-old infant experienced severe chloramphenicol toxicity associated with high serum levels (313 micrograms/ml). Cardiovascular collapse with cardiomyopathic changes and impaired left ventricular function was documented by echocardiography. Serial echocardiographic evaluation showed resolution of the cardiomyopathic findings as the chloramphenicol levels were spontaneously cleared. Clinical course was complicated by the development of liver disease and coagulopathy compatible with disseminated intravascular consumption. Patient's recovery was complete and uneventful, nevertheless, chloramphenicol toxicity in childhood is associated with a significant mortality rate of 40%. The related impaired cardiac function, although reversible, appears to play a major role in the pathogenesis and eventual outcome in this syndrome.- - - - - - - - - - ranking = 0.0045338842630118keywords = dysfunction (Clic here for more details about this article) |
6/8. Onset of right-to-left shunting through a foramen ovale in a 70-year-old woman: successful surgical treatment.A 70-year-old woman presented with disabling breathlessness. She was found to have severe arterial hypoxaemia due to isolated right-to-left shunting through a patent foramen ovale. In the absence of pulmonary hypertension or evidence of right ventricular dysfunction this is attributed to reduced right atrial compliance. The phasic nature of the shunt, which occurred only during atrial filling, supports this view. Surgical closure of the foramen abolished the hypoxaemia and her symptoms.- - - - - - - - - - ranking = 0.5keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
7/8. Recognizing congestive heart failure in the neonatal period.Signs of congestive heart failure can be subtle and vary in the time frame of presentation. As a result, this diagnosis is often missed in its early stages. This article reviews risk factors for myocardial dysfunction in the newborn period. Additionally, there is a comprehensive overview of cardiac physiology as it relates to the presentation of symptoms of congestive heart failure. This information is presented to provide a foundation of knowledge related to physiologic changes involved in congestive heart failure so that the health care provider can recognize congestive heart failure early.- - - - - - - - - - ranking = 0.0011334710657529keywords = dysfunction (Clic here for more details about this article) |
8/8. Resolution of cavopulmonary shunt-associated pulmonary arteriovenous malformation after heart transplantation.A child with heterotaxia, azygous continuation of an interrupted inferior vena cava, single ventricle, and pulmonary atresia underwent the Kawashima modification of the fontan procedure, which excluded hepatic venous return from the pulmonary blood flow. After the operation, the patient had development of pulmonary arteriovenous malformations, increasing cyanosis, and ventricular dysfunction. He underwent orthotopic heart transplantation at 7 years of age with prompt resolution of the pulmonary arteriovenous malformations.- - - - - - - - - - ranking = 0.5keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |