[Clinico-pathological characteristics of infective endocarditis associated with congenital heart disease: an analysis of 74 cases in single center].

OBJECTIVE: To assess the changing profile of infective endocarditis (IE) in patients with congenital heart disease (CHD) from 1998 to 2009 in our hospital. METHODS: Clinical characteristics of IE patients with CHD underwent surgical treatment during 1998 - 2009 in our hospital were evaluated. The coincidence rate between clinical and pathological diagnosis were analyzed. RESULTS: There were 74 IE cases associated with CHD during the 12 years, accounting for 33.6% of all patients with IE receiving surgery during this time period. Mean age was higher for patients treated in 2006 - 2009 than patients treated in 2002 - 2005 [(38.7 ± 14.6) years vs. (28.4 ± 12.8) years, P = 0.003].Bicuspid aortic valve (accounting for 52.2%) was the most frequent congenital heart disease and the age of these patients was younger than patients with other congenital heart diseases. IE in CHD affected the left heart structures in 83.8% (62/74) of all cases, 47 in aortic valve (75.8%). Blood cultures were performed in 29.7% of the patients (22/74) and the positive rate was 59.1% (13/22). Streptococci viridans were the most common causative organisms. Echocardiography was performed in all patients and 66.3% echocardiographic records were positive, IE was diagnosed in 53 patients (71.7%) before operation. The operative mortality was 2.7%. CONCLUSION: Congenital heart disease, especially bicuspid aortic valve, is the most common underlying disease for IE. Combined analysis of clinical, echocardiographic and blood culture results are essential for increasing the diagnosis rate of IE.

[Clinical implication of microvasculopathy in patients post heart transplantation].

OBJECTIVE: Assess the clinical implication of microvasculopathy detected by endomyocardial biopsy samples in patients post heart transplantation. METHODS: Light microscopic evaluations were performed in 278 endomyocardial biopsies harvested from 64 patients post heart transplantation for more than one year, microvasculopathy was defined as stenotic endothelial and/or medial disease. RESULTS: The patients with stenotic microvasculopathy were younger than those without microvasculopathy (40.7 ± 15.9 vs. 49.4 ± 8.7, P < 0.05). The mean score of acute cellular rejection (0.83 ± 0.39 vs. 0.37 ± 0.32, P < 0.01) and the numbers of ≥ grade II acute rejection (0.84 ± 0.16 vs. 0.23 ± 0.10, P < 0.01) were significantly greater in stenotic microvasculopathy group compared to those of non-stenotic group. Multivariate regression analysis confirmed that stenotic microvasculopathy is the independent risk factor for the mean acute rejection score (OR = 3.40, 95%CI, 4.62 - 193.07, P < 0.01), but not for the Quilty lesion, coronary heart disease of donor, diabetes mellitus. Angiographically confirmed coronary vasculopathy and cardiac dysfunction (χ(2) = 0.94, P > 0.05 and χ(2) = 2.90, P > 0.05) were similar between microvasculopathy group and non-microvasculopathy group. CONCLUSION: Post heart transplantation microvasculopathy is an immune-mediated phenomenon and associated with higher mean score of acute cellular rejection and higher numbers of ≥ grade II acute rejection but was not the prognostic risk factor for coronary vasculopathy and function reduction after heart transplantation.

Prevalence and Clinical Characteristics of Fabry Disease in Chinese Patients With Hypertrophic Cardiomyopathy.

BACKGROUND: The prevalence of Fabry disease (FD) in Chinese patients with hypertrophic cardiomyopathy (HCM) is unclear. We aimed to evaluate the prevalence, clinical characteristics, and outcomes of FD in Chinese patients with HCM. METHODS: Of 217 patients with HCM, FD probands were screened by next-generation sequencing at Fuwai Hospital. Medical data from α-galactosidase A activity, electrocardiography, echocardiography, coronary angiography, cardiac magnetic resonance, pathological examination, and follow up was analyzed. RESULTS: Two FD probands were observed (0.93% of patients with HCM), both of which were diagnosed with symptomatic obstructive HCM at 49 years of age. One proband had a GLA mutation (c.887T>C [p.M296T]) with a late-onset cardiac variant, which was characterized by dual ventricular hypertrophy and conduction disease with a permanent pacemaker. The other patient had a GLA mutation (c.758T>C [p.I253T]) with a classic phenotype and dual ventricular hypertrophy, atrioventricular block, renal failure, and recurrent cerebral infarction. Both probands had late gadolinium enhancement mainly in the basal segment of the inferolateral wall. Follow up revealed no exertional symptoms or outflow obstruction after surgical septal myectomy in the two probands, and stable renal function was observed after 6 months of migalastat therapy in the later one. A family study revealed six female carriers and three sudden cardiac deaths. CONCLUSIONS: FD is not uncommon in Chinese patients with HCM. Multiple organic involvement, dual ventricular hypertrophy, and conduction disease provide clinical clues for suspected FD, and early genetic screening is necessary. Surgical septal myectomy and migalastat improve the long-term prognosis of patients with FD.

[Clinicopathologic analysis of dilated heart in cardiac transplant recipients].

OBJECTIVE: To study the pathologic features of dilated heart in cardiac transplant recipients, with clinicoradiologic correlation. METHODS: Sixty recipient hearts from cardiac transplantation performed in Fuwai Hospital were analyzed by gross examination, histologic observation and electron microscopy. Clinicoradiologic correlation was available in 40 cases. RESULTS: Amongst the 40 cases of dilated heart, 52.5% (21/40) were due to dilated cardiomyopathy, 22.5% (9/40) due to arrhythmogenic right ventricular cardiomyopathy, 15.0% (6/40) due to ischemic cardiomyopathy, and the remaining 10.0% (4/40) due to miscellaneous causes, including local noncompaction of ventricular myocardium, giant cell myocarditis, alcoholic cardiomyopathy and hypertensive cardiomyopathy. The discrepancy rate between clinical and pathologic diagnosis was 37.5% (15/40). The erroneous categories included arrhythmogenic right ventricular cardiomyopathy (7 cases), ischemic cardiomyopathy (5 cases), and giant cell myocarditis (1 case), which were all mistaken clinically as dilated cardiomyopathy. While ischemic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, noncompaction of ventricular myocardium and giant cell myocarditis have distinctive pathologic features, the diagnosis of alcoholic and hypertensive cardiomyopathies required clinicopathologic correlation. Dilated cardiomyopathy due to viral myocarditis was not identified in the cases studied. CONCLUSION: Pathologic examination is essential in analysis of transplant recipient heart and helps to rectify clinical diagnostic discrepancy.