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1/12. Clinical and pathologic study of two siblings with arrhythmogenic right ventricular cardiomyopathy.

    ARVC is a cardiomyopathy in which the right ventricular myocardium is replaced by fibroadipose tissue. Males are affected slightly more often than females and, in those cases which are familial, the pattern of inheritance is usually autosomal dominant with incomplete penetrance. We examined the hearts of two sisters, ages 17 and 14, with no family history of heart disease. The older sibling, who was previously considered healthy, died suddenly, while the younger sibling developed congestive heart failure and received a cardiac transplant. An autopsy of the older sibling and examination of the younger sibling's excised heart revealed severe examples of ARVC with minor differences. A thick cap of fibroadipose tissue covered most, if not all, of each right ventricle and was transmural in some areas. Microscopically, lelt ventricles contained extensive myocyte disarray and multifocal fibrosis. The coronary arteries displayed intimal hyperplasia with disruption of the internal elastic lamina, similar to fibromuscular dysplasia. These two cases comprise a unique familial grouping in a polymorphic disease. Despite the male predominance and autosomal dominant inheritance in ARVC, the only members affected in this family were female, and an autosomal dominant pattern of inheritance, even with incomplete penetrance, would be unusual. In addition, we identified changes in the coronary arteries similar to fibromuscular dysplasia and corroborated recently reported changes in the left ventricle of patients with ARVC, providing evidence that this disease, in its most severe form, involves the entire heart.
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2/12. Absent pulmonary valve with tricuspid atresia or severe tricuspid stenosis: report of three cases and review of the literature.

    Absence of the pulmonary valve occurs usually in association with tetralogy of fallot and occasionally with an atrial septal defect or as an isolated lesion. Very rarely it occurs with tricuspid atresia, intact ventricular septum, and dysplasia of the right ventricular free wall and of the ventricular septum. We present the clinical, anatomic, and histologic findings of a new case, and for the first time, the data from two patients with absent pulmonary valve and severe tricuspid stenosis, who exhibited similar histologic findings. We also reviewed the clinical and anatomic data of 24 previously published cases and compared them with the new cases. In all three new cases, the myocardium of the right ventricle was very abnormal. In the two cases with tricuspid stenosis, large segments of myocardium were replaced with sinusoids and fibrous tissue. In the case with tricuspid atresia, the right ventricular free wall contained only fibroelastic tissue. The ventricular septum in all three patients showed asymmetric hypertrophy and in two of the three patients, multiple sinusoids had replaced large segments of myocardial cells. The left ventricular free wall myocardium and the walls of the great arteries were unremarkable. Our data indicate that myocardial depletion involving the right ventricular free wall and the ventricular septum and its replacement by sinusoids and fibroelastic tissue occur not only in cases of absent pulmonary valve with tricuspid atresia but also in cases of absent pulmonary valve with tricuspid stenosis. The degree of myocardial depletion varies and is more severe when the tricuspid valve is atretic.
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3/12. Balloon pulmonary valvuloplasty in carcinoid syndrome.

    Half of all patients with carcinoid syndrome develop cardiac involvement. patients who have cardiac involvement have a significantly worse prognosis than those without, and death can occur directly as a result of cardiac involvement. A case of carcinoid syndrome in a 38 year old woman with lesions in the liver, who presented with right sided valvar abnormalities, a dilated right ventricle, and right ventricular pressure overload, is presented. In order to palliate the patient's symptoms and to decrease right sided pressures before major abdominal surgery, balloon pulmonary valvuloplasty was performed at the time of cardiac catheterisation. This resulted in a reduction in the pulmonary gradient and right ventricular pressure. Following the procedure, the patient's symptoms were completely relieved. She went on to laparotomy where the lesions in the liver were excised without complication.
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4/12. Right ventricular involvement in hypertrophic cardiomyopathy: a case report and literature review.

    Although hypertrophic cardiomyopathy (HCM) is classically considered a disease of the left ventricle, right ventricular (RV) abnormalities have also been reported. However, involvement of the right ventricle in HCM has not been extensively characterized. The literature regarding prevalence, genetics, patterns of involvement, histologic findings, symptoms, diagnosis, and treatment of RV abnormalities in HCM is reviewed. To highlight the salient points, a case is presented of apical HCM with significant RV involvement, with an RV outflow tract gradient and near obliteration of the RV cavity, in the absence of a left intraventricular gradient. Right ventricular involvement in HCM appears to be as heterogeneous as that of the left ventricle. The spectrum extends from mild concentric hypertrophy to more unusual severe, obstructive disease. While in some cases the extent of RV involvement correlates with left ventricular (LV) involvement, predominant RV disease can be seen as well. While the genetics of RV involvement have not been well characterized, histologic findings appear to be similar to those in the left ventricle, suggesting similar pathogenesis. Significant RV involvement may result in RV outflow obstruction and/or reduced RV diastolic filling, with potentially increased incidence of severe dyspnea, supraventricular arrhythmias, and pulmonary thromboembolism. The optimal treatment for patients with significant RV disease is unknown. Medical and surgical therapies have been attempted with variable success; experience with newer techniques such as percutaneous catheter ablation has not been reported. Further characterization of RV involvement in HCM is necessary to elucidate more clearly the clinical features and optimal treatments of this manifestation of HCM.
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5/12. Isolated right ventricular hypertrophic obstructive cardiomyopathy.

    Two cases of isolated hypertrophic cardiomyopathy of right ventricle without any involvement of interventricular septum or left ventricle are reported. Two cases reported in literature are also reviewed. In these cases symptoms are those of left ventricular hypertrophic obstructive cardiomyopathy. Right ventricular dominance on clinical examination and electrocardiogram and inspiratory increase in intensity of murmur are helpful clues. 2-Dimensional echocardiography with Doppler evaluation can confirm the diagnosis noninvasively.
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6/12. Acquired right heart outflow tract anomaly without systemic hypertension in recipient twins in twin-twin transfusion syndrome.

    Significant hemodynamic changes are commonly observed in both fetuses in twin-twin transfusion syndrome. In the recipient twin there is cardiac dysfunction with hypertrophy of both ventricles and overall enlargement of the heart. We describe five cases of recipient twins in twin-twin transfusion syndrome with right ventricle hypertrophy, pulmonary stenosis and tricuspid regurgitation acquired in utero. These symptoms developed with no signs of systemic hypertension. Three of the five recipient twins survived and were developing normally at 3-30 months of age. Postnatal outcome is likely to have improved as a result of prenatal diagnosis of right ventricle outflow tract obstruction and timed delivery. These acquired anomalies of the right heart might be related to the particular hemodynamic conditions of the recipient twin. A global heart dilatation is logically expected, but this hypertrophy without dilatation is probably related to the shared plasma of fetuses with opposite hemodynamic conditions. This is what we consider as the hemodynamic-hormonal paradox. These acquired anomalies, though severe, are accessible to neonatal treatment if treated early.
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7/12. Transient severe isolated right ventricular hypertrophy in neonates.

    We report two instances of transient isolated right-sided myocardial hypertrophy in patients with an intact ventricular septum, normal thickness of the posterior wall of the left ventricle, and normal ventricular function, diagnosed by echocardiography on the third day of life. The two neonates, born at 36 and 38 weeks gestation respectively, had perinatal distress. Both were diagnosed as having isolated right ventricular hypertrophy with mild pulmonary hypertension, which disappeared in both cases within 8 weeks without any specific therapy. Though the cause of the ventricular hypertrophy remains unclear, we believe that it is the consequence of remodeling of pulmonary vasculature secondary to acute perinatal distress, resulting in persistent pulmonary hypertension and producing pressure overload on the right ventricle, and hence right ventricular hypertrophy. The finding of early and transient right ventricular hypertrophy, with normal left-sided structures and normal ventricular function, has thus far failed to gain attention in the paediatric cardiologic literature.
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8/12. Noncompacted myocardium in Ebstein's anomaly: initial description in three patients.

    Exercise intolerance in Ebstein's anomaly is usually attributed to desaturation secondary to right-to-left shunting as a result of a small or distorted left ventricle (LV), significant tricuspid valve regurgitation, right ventricular dysfunction, or a combination of these. We observed one boy (age 15 years) and two women (ages 20 and 29 years) with severe Ebstein's anomaly and strikingly abnormal LV myocardium resembling the features described for LV noncompaction. LV size and systolic function were normal in the two women; the boy had a dilated LV with severely diminished ejection fraction. The LV myocardium was found to be unusually coarse and hypertrabeculated, with small intertrabecular recesses and an irregular endocardial surface. The findings in these 3 patients represent the whole spectrum of mild to severe LV noncompaction. Diastolic dysfunction was present in 2 of the 3 patients. exercise tolerance was diminished in all. There was no mitral or aortic valve disease. The 15-year-old boy underwent heart transplantation 6 months later for biventricular failure. Thus, Ebstein's anomaly does not seem to be a pathology confined to the right ventricle, but may rarely lead to LV noncompacted myocardium. This LV pathology may be an additional explanation for exercise intolerance or signs of left heart failure in patients with Ebstein's anomaly.
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9/12. Two cases of familial primary pulmonary hypertension.

    Case 1, a 28-year-old woman (third daughter of Case 2) delivered her first child in September 2000, but leg edema and dyspnea on exertion appeared the following January. At the time of our first examination of the patient in May 2001, a chest X-ray showed cardiomegaly and pulmonary artery enlargement. echocardiography demonstrated enlargement of the right ventricle and small left ventricular dimensions, and an electrocardiogram revealed right ventricle hypertrophy. After perfusion-ventilation lung scintigraphy and cardiac catheterization, she was diagnosed as having primary pulmonary hypertension (PPH). Although she was discharged with prescriptions for a diuretic, warfarin and beraprost sodium, she died of a pulmonary hypertensive crisis twenty days after readmission. Case 2, a 60-year-old woman(mother of Case 1) developed the same symptoms as those in Case 1, in May 2001, but recovered after medication. PPH is a rare disease and only a few familial cases are reported. In this family, the eldest daughter of Case 2 had also died of pulmonary hypertension ten years ago, several months after her first delivery. In contrast to the daughters, the mother's symptoms developed gradually.
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10/12. Right ventricular enlargement mimicking electrocardiographic left ventricular pacing.

    Electrocardiographic right bundle branch block morphology during cardiac pacing is occasionally the result of accidental placement of pacemaker or defibrillator leads into the left ventricle. Inadvertent lead placement in the left heart is associated with a risk of systemic embolism. Previous authors have attempted to define safe (right ventricular origin) and unsafe (left ventricular origin) patterns of right bundle branch block during pacing. We report a case of a patient with severe dilated cardiomyopathy and a correctly positioned pacemaker-defibrillator lead in the right ventricular apex, who meets electrocardiographic criteria for lead implantation into the left ventricle.
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