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Suicide right ventricle after lung transplantation for Eisenmenger syndrome.

Right ventricular outflow tract obstruction, or "suicide right ventricle," rarely has been observed after single or bilateral single-lung transplantation for the treatment of Eisenmenger syndrome. We describe our experience in 2 patients with Eisenmenger syndrome in whom right ventricular outflow tract obstruction developed, in 1 after single-lung transplantation and ventricular septal defect repair and in the other after bilateral single-lung transplantation. Both patients suffered progressive deterioration and hemodynamic instability that was unresponsive to aggressive medical therapy. Diagnosis was confirmed in both patients by transesophageal echocardiography. Operative intervention was undertaken 72 and 24 hours after transplantation, and consisted of myectomy and outflow tract patching. One patient survived; the other died intraoperatively. The index of suspicion for this problem should be high during the intraoperative performance of transesophageal echocardiography, as well as during direct gradient measurement, with consideration of immediate management of severe right ventricular outflow tract obstruction at the time of transplantation.

Adult↗

Pulmonary hypertension associated with COPD.

Pulmonary hypertension is a common complication of chronic obstructive pulmonary disease (COPD). The increase in pulmonary artery pressures is often mild to moderate, but some patients may suffer from severe pulmonary hypertension, and present with a progressively downhill clinical course because of right-sided heart failure added to ventilatory handicap. The cause of pulmonary hypertension in COPD is generally assumed to be hypoxic pulmonary vasoconstriction leading to permanent medial hypertrophy. However, recent pathological studies point, rather, to extensive remodeling of the pulmonary arterial walls, with prominent intimal changes. These aspects account for minimal reversibility with supplemental oxygen. There may be a case for pharmacological treatment of pulmonary hypertension in selected patients with advanced COPD and right-sided heart failure. Candidate drugs include prostacyclin derivatives, endothelin antagonists and inhaled nitric oxide, all of which have been reported of clinical benefit in primary pulmonary hypertension. However, it will be a challenge for randomized controlled trials to overcome the difficulties of the diagnosis of right ventricular failure and the definition of a relevant primary endpoint in pulmonary hypertensive COPD patients.

Humans↗

Repair of isolated multiple muscular ventricular septal defects: the septal obliteration technique.

BACKGROUND: Isolated multiple ventricular septal defects (mVSDs) remain a surgical challenge. The dilemma of whether to perform a complete repair ultimately rests with the surgeon, who must decide if all significant septal defects can be located. Avoidance of a pulmonary arterial band (as part of a two-stage repair) will negate the need for future pulmonary arterial reconstruction and will reduce the incidence of late right ventricular diastolic dysfunction. METHODS: We performed a retrospective analysis of hospital and echocardiographic data of eight children who underwent a septal obliteration technique (SOT) as part of their correction of mVSDs (with and without coarctation of the aorta). RESULTS: Eight children with a mean age of 10.5 months (range 1.5 to 36 months), and weight of 6.2 kg (range 2.1 to 13.5 kg), respectively, underwent correction of mVSDs. All had a single, large, perimembranous defect, additional VSDs within the muscular trabecular septum (juxtaposed to the moderator band), and apical mVSDs. All VSDs were repaired via the right atrium, with avoidance of either a right or left ventriculotomy. The posterior and apical defects were excluded from the right ventricular cavity with a pericardial patch (SOT). The follow-up period remains limited to a mean of 20.9 months (8 to 39 months). Two children repaired with SOT had previous pulmonary artery bands (neonatal coarctation repair). All children were successfully discharged home with a mean postoperative Qp:Qs of 1.09:1. One pacemaker was required, but this child has since reverted back to normal sinus rythm. CONCLUSIONS: Our initial experience using the SOT in the treatment of apical VSDs as a component of isolated mVSDs has been rewarding. All children are currently alive, in normal sinus rhythm, and have no residual significant left-to-right shunts.

Cardiac Surgical Procedures↗

Doppler tissue imaging analysis of ventricular function after surgical and transcatheter closure of atrial septal defect.

The long-term ventricular function of children after surgical and transcatheter closure of secundum atrial septal defects was assessed by Doppler tissue imaging and compared with that of age-matched controls. Long-term right ventricular function was impaired in patients after surgical repair, as reflected by decreased systolic and diastolic tricuspid annular velocities and an increased Tei index. In contrast, right ventricular function was preserved after transcatheter device closure.

Adolescent↗

[Reagudization of Chagas myocarditis inducing exclusive right ventricular failure].

Woman, 42 years-old, receiving immunosuppressive therapy for a lymphoma, presented reagudization of Chagas' disease, from its indeterminate phase. Intense inflammatory visceral aggression, due to extensive intracellular proliferation of the Trypanosoma cruzi, was the likely mechanism for acute myocarditis leading to severe right ventricular failure. Antiparasite chemotherapy was effective in the control of visceral involvement and for the remission of cardiac failure. The clinical course in this case is compatible with the hypothesis of early right ventricular damage in Chagas' disease.

Adult↗

[Right precordial leads and sudden death. Relation with arrhythmogenic right ventricular dysplasia].

Non-coronary ST-segment elevation during right sided chest pain has been described in subjects with episodes of ventricular fibrillation at rest. This syndrome has been attributed to functional phenomena or to structural myocardial changes. A personal case has features belonging to two categories: ST-segment elevation observed before, during and after episodes of arrhythmia was compared to 11 previously recorded ECG recordings. Right ventricular dysplasia was shown by electrocardiography, electrophysiology and echocardiography. In addition, ST-segment elevation is classified in 3 categories: triangular and dome-shaped are the most commonly observed forms during the arrhythmias: the third form with "saddle"-shaped appearances has not been previously described and would seem to be a minor equivalent observed during intercritical periods. This form is found in 30% of clinically documented cases of arrhythmogenic right ventricular dysplasia.

Arrhythmias, Cardiac↗

Myocardial bridging confined to the right ventricular branch of the right coronary artery in a patient with severe pulmonary hypertension.

Although myocardial bridges are mostly confined to the left anterior descending coronary artery, several cases of right coronary artery myocardial bridging have been reported in the literature. In the current case report, we present a 65-year-old female with a well-functioning mechanical mitral valve prosthesis, severe pulmonary hypertension and right ventricle wall motion abnormality in whom diagnostic angiography revealed myocardial bridging confined to the right ventricular branch of the right coronary artery.

Aged↗

Rare variants of divided right ventricle with sequestered apical trabecular component.

The right ventricle may be divided into two or more compartments by various structures in various ways. Rarely, the apical trabecular component may be sequestered from the rest of the right ventricle. We report 4 cases with different underlying lesions that share a common pathology of apical sequestration of the right ventricle resulting in diverse hemodynamic consequences. Case 1 had pulmonary valve stenosis. The apical sequestration of the right ventricle resulted in no significant hemodynamic consequence. Case 2 had multiple defects in the muscular ventricular septum. The volume of left-to-right shunt seemed to be reduced because of the commitment of some of the defects to the sequestered cavity. Case 3 had a large defect in the trabecular septum. As the defect involved the whole septum that was related to the sequestered right ventricular apex, the left ventricle together with the sequestered right ventricle formed a boot-shaped chamber. Hemodynamically, the muscular shelf was an interventricular septum. Case 4 had a coronary artery fistula to an isolated cavity that occupied the apical region of the right ventricle. The pathology was similar to the case that was reported as a five-chambered heart. The abnormal cavity was, in fact, the sequestered right ventricular apex.

Adolescent↗