Leaflet escape in a new bileaflet mechanical valve: TRI technologies.

Background: Leaflet escape is a mode of structural valve failure for mechanical prostheses. This complication previously has been reported for both monoleaflet and bileaflet valve models. We report 2 leaflet escape occurrences observed in 2 patients who underwent valve replacement with a TRI Technologies valve prosthesis.

Coronary thrombosis and sudden death after an enteroviral infection. Case report.

We report the case of a 26-year-old man who died suddenly 9 days after an episode of flu. Microscopic examination of the left anterior descending coronary artery showed an eccentric fibroatheromatic plaque complicated by thrombosis, endothelial erosion and extensive T-cell and macrophage infiltration. Frozen sections of the thrombotic coronary segment, analysed for different infective agents by polymerase chain reaction (PCR) and reverse transcriptase (RT)-PCR, showed positive amplification for an enteroviral genome.

Immunosuppressive therapy for active lymphocytic myocarditis: virological and immunologic profile of responders versus nonresponders.

Background: The beneficial effect of immunosuppressive treatment on myocarditis is still controversial, possibly because the immunologic and virological profile of potential candidates is largely unknown.

Methods And Results: Out of 652 biopsied patients, 112 had a histological diagnosis of active lymphocytic myocarditis; 41 of these 112 patients were characterized by progressive heart failure despite conventional therapy and were treated with prednisone and azathioprine for 6 months. All were resubmitted to cardiac catheterization, angiography, and endomyocardial biopsy at 1 and 6 months and followed-up for 1 year.

Successful treatment of enterovirus-induced myocarditis with interferon-alpha.

No randomized, placebo-controlled studies have investigated interferon-alpha therapy in enterovirus-proven myocarditis. This report describes 2 patients with enterovirus-induced myocarditis (1 with associated Churg-Strauss syndrome) who at follow-up endomyocardial biopsy showed clinical and hemodynamic improvement and viral clearance (using polymerase chain reaction) after interferon-alpha therapy.

Arrhythmogenic right ventricular cardiomyopathy type 1 (ARVD1): confirmation of locus assignment and mutation screening of four candidate genes.

Arrhythmogenic right ventricular cardiomyopathy type 1 (ARVD1) is an autosomal dominant disorder characterised by progressive degeneration of right ventricular myocardium, arrhythmias and risk of sudden death. By linkage analysis, we previously mapped the involved gene to chromosome 14q24.3.

Recurrence of idiopathic pulmonary hemosiderosis in a young adult patient after bilateral single-lung transplantation.

Background: Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology characterized by hemoptysis, diffuse pulmonary infiltration, and anemia. Diagnosis requires a detailed clinical history and transbronchial lung biopsy (TLB).

Methods And Results: A 19-year-old man developed progressive dyspnea, hemoptysis, and anemia.

Molecular diagnosis of myocarditis and dilated cardiomyopathy in children: clinicopathologic features and prognostic implications.

Myocarditis is the most common cause of heart failure in children. We investigated viral etiology of myocarditis/dilated cardiomyopathy (DCM) in children and correlated molecular findings with pathologic and clinical data. Polymerase chain reaction (PCR) or reverse transcription (RT)-PCR were used to analyze 59 endomyocardial biopsies from 48 consecutive young (<18 yrs) patients (pts) with clinical and histologic diagnosis of myocarditis and DCM, employing primers designed to amplify specific sequences of various DNA and RNA viruses.

Heart rate variability in arrhythmogenic right ventricular cardiomyopathy correlation with clinical and prognostic features.

The identification of subjects with arrhythmogenic right ventricular cardiomyopathy (ARVC) at higher risk for sudden death is an unresolved issue. An influence of the autonomic activity on the genesis of ventricular arrhythmias was postulated. Heart rate variability (HRV) analysis provides a useful method to measure autonomic activity, and is a predictor of increased risk of death after myocardial infarction.

Lone hepatitis C virus myocarditis responsive to immunosuppressive therapy.

Objectives: This study analyzes the causal role of hepatitis C virus (HCV) in patients with lone myocarditis, and its susceptibility to immunosuppression.

Background: Prevalence of HCV in lone myocarditis, its mechanism of damage, and possible treatment are still unknown.

Methods: Among 48 consecutive patients with myocarditis serologically screened for HCV and other cardiotropic viruses, 3 patients had anti-HCV antibodies.

Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy.

Arrhythmogenic right ventricular cardiomyopathy (ARVD/C) is a genetically heterogeneous disease characterized by progressive degeneration of the right ventricular myocardium and increased risk of sudden death. Here, we report on a genome scan in one Italian family in which the disease appeared unlinked to any of the six different ARVD loci reported so far; we identify a mutation (S299R) in exon 7 of desmoplakin (DSP), which modifies a putative phosphorylation site in the N-terminal domain binding plakoglobin. It is interesting that a nonsense DSP mutation was reported elsewhere in the literature, inherited as a recessive trait and causing a biventricular dilative cardiomyopathy associated with palmoplantar keratoderma and woolly hairs.

Left main trunk ostial stenosis and aortic incompetence in Takayasu's arteritis.

A 41-year-old woman with recent onset of heart failure and angina due to aortic valve incompetence and critical left coronary ostium stenosis in the setting of Takayasu's arteritis is reported. The patient was successfully surgically treated by aortic valve replacement and coronary artery bypass with saphenous vein graft, showing a cardiac event-free 17 months follow-up. Takayasu's arteritis must be included among the possible causes of coronary artery disease and aortic valve incompetence in young female patients.

Biodegradable polymer (D,L-lactide-epsilon-caprolactone) in aortic vascular prosthesis: morphological evaluation in an animal model.

Biodegradable D,L-Lactide-epsilon-caprolactone copolymer was used in substitution to bovine collagen to seal porosity in nine Dacron vascular Sorin Carbografts. One served as control and 8 were implanted in mini-pigs as vascular by-pass in the thoracic aorta. The grafts were explanted at 7 days (4 animals), 30 (2 animals) and at 90 days (2 animals), and submitted to gross examination, X-ray, histology and electron microscopy.

Unexpected mechanical bileaflet valve thrombosis in mitral position: what is better to do, re-replacement or thrombolysis.

Background And Aims: The longevity of the mechanical heart valve prosthesis is an advantage when compared with its biological counterpart, although the former carries a risk of thrombosis depending on valve design, materials and host-related interface; therefore, a patient with a mechanical valve prosthesis, particularly in mitral position, is at risk for systemic thromboembolism even when properly anticoagulated.

Patients And Methods: We report a case of a 60-year-old woman who underwent a mitral valve replacement with a St. Jude Medical (SJM) standard bileaflet mechanical heart valve.

Fatal outcome in Eisenmenger syndrome.

Aim: To assess correlations between fatal outcome and histologic findings of pulmonary vascular disease in different situations of Eisenmenger syndrome, either during the natural course or early-late after surgery.

Material And Methods: The clinical follow-up and fatal outcome of 20 patients affected by Eisenmenger syndrome were investigated. In addition to the pathologic report and gross reexamination of the heart, the lung tissue was studied by histology.

Clefted bicuspid aortic valve.

We report an unusual case of bicuspid aortic valve in a 67-year-old man who had aortic valve replacement for combined severe aortic stenosis with moderate aortic regurgitation. Both preoperative echocardiography and angiocardiography failed to recognize the bicuspid nature of the aortic valve, which was discovered only at surgical pathology examination. The atypical anatomic findings of the valve consisted of a "cleft-like" appearance of the ventral leaflet formed by the two conjoined cusps together with a fenestrated rudimental raphe.

Directional atherectomy of a calcified lesion using a new atherectomy device.

We report on a case of directional atherectomy performed on a calcified coronary lesion using a novel device with a hardened titanium cutter. The successful removal of calcified plaque was documented by intravascular ultrasound assessment and confirmed by histopathological analysis of the obtained plaque specimen.

Screening for ryanodine receptor type 2 mutations in families with effort-induced polymorphic ventricular arrhythmias and sudden death: early diagnosis of asymptomatic carriers.

Objectives: We sought to establish the role of genetic screening for ryanodine receptor type 2 (RyR2) gene mutations in families with effort-induced polymorphic ventricular arrhythmia (PVA), syncope and juvenile sudden death.

Background: The RyR2 mutations have been associated with PVA, syncope and sudden death in response to physical or emotional stress.

Methods: We studied 81 subjects (39 males and 42 females; mean age 31 +/- 20 years) belonging to eight families with pathogenic RyR2 mutations.

Endothelin A-receptor antagonist administration immediately after experimental myocardial infarction with reperfusion does not affect scar healing in dogs.

Objective: Endothelin (ET) receptor antagonists have been reported to reduce both infarct size and no-reflow phenomenon; however, in rat models their effect on the healing process after myocardial infarction (MI) is controversial. The study aimed to evaluate the effect of early administration of the ET(A) receptor antagonist darusentan on scar healing in an ischemia-reperfusion model in dogs.

Methods: Thirty male mongrel dogs surviving 180 min left anterior descending coronary artery balloon occlusion were randomised to: darusentan i.

Bicuspid aortic valve: abnormal aortic elastic properties.

Background And Aims Of The Study: Bicuspid aortic valve (BAV) is frequently associated with clinically relevant abnormalities of the aorta, suggesting the existence of a common underlying developmental defect involving the aortic valve and wall of the ascending aorta. The study aim was to evaluate noninvasively the elastic properties of the aortic root in young males with BAV, to discover whether structural abnormalities of the aorta might be manifested by impairment in elasticity.

Methods: Forty-nine young male subjects with isolated BAV were consecutively detected during preenrollment military screening, and studied using transthoracic echocardiography.