Outcome after aortic valve replacement in children: A systematic review and meta-analysis.

OBJECTIVE: Despite an increasing interest in pediatric aortic valve repair, aortic valve replacement in children may be unavoidable. The evidence on outcome after pediatric aortic valve replacement is limited and usually reported in small case series. This systematic review and meta-analysis aims to provide an overview of reported outcome of pediatric patients after aortic valve replacement. METHODS: A systematic literature search for publications reporting outcome after pediatric aortic valve replacement published between January 1990 and May 2015 was conducted. Studies written in English with a study size of more than 30 patients were included. RESULTS: Thirty-four publications reporting on 42 cohorts were included in this review: 26 concerning the Ross procedure (n = 2409), 13 concerning mechanical prosthesis aortic valve replacement (n = 696), and 3 concerning homograft aortic valve replacement (n = 224). There were no studies on bioprostheses that met our inclusion criteria. The pooled mean patient age was 9.4 years, 12.8 years, and 8.9 years for Ross, mechanical prosthesis, and homograft recipients, respectively. Pooled mean follow-up was 6.6 years. The Ross procedure was associated with lower early (4.20%; 95% confidence interval [CI], 3.37-5.22 vs 7.34%; 95% CI, 5.21-10.34 vs 12.82%; 95% CI, 8.91-18.46) and late mortality (0.64%/y; 95% CI, 0.49-0.84 vs 1.23%/y; 95% CI, 0.85-1.79 vs 1.59%/y; 95% CI, 1.03-2.46) compared with mechanical prosthesis aortic valve replacement and homograft aortic valve replacement, respectively. No significantly different aortic valve reoperation rates were observed between the Ross procedure and mechanical prosthesis aortic valve replacement (1.60%/y; 95% CI, 1.27-2.02 vs 1.07%/y; 95% CI, 0.68-1.68, respectively), whereas homograft aortic valve replacement was associated with significantly higher aortic valve reoperation rates (5.44%/y; 95% CI, 4.24-6.98). The Ross procedure-associated right ventricular outflow tract reoperation rate was 1.91% per year (95% CI, 1.50-2.44). CONCLUSIONS: This systematic review illustrates that all currently available aortic valve substitutes are associated with suboptimal results in children, reflecting the urgent need for reliable and durable repair techniques and innovative replacement solutions for this challenging group of patients.

Ventricular tachyarrhythmia during pregnancy in women with heart disease: Data from the ROPAC, a registry from the European Society of Cardiology.

OBJECTIVES: To describe the incidence, onset, predictors and outcome of ventricular tachyarrhythmia (VTA) in pregnant women with heart disease. BACKGROUND: VTA during pregnancy will cause maternal morbidity and even mortality and will have impact on fetal outcome. Insufficient data exist on the incidence and outcome of VTA in pregnancy. METHODS AND RESULTS: From January 2007 up to October 2013, 99 hospitals in 39 countries enrolled 2966 pregnancies in women with structural heart disease. Forty-two women (1.4%) developed clinically relevant VTA during pregnancy, which occurred mainly in the third trimester (48%). NYHA class >1 before pregnancy was an independent predictor for VTA. Heart failure during pregnancy was more common in women with VTA than in women without VTA (24% vs. 12%, p=0.03) and maternal mortality was respectively 2.4% and 0.3% (p=0.15). More women with VTA delivered by Cesarean section than women without VTA (68% vs. 47%, p=0.01). Neonatal death, preterm birth (<37weeks), low birthweight (<2500g) and Apgar score <7 occurred more often in women with VTA (4.8% vs. 0.3%, p=0.01; 36% vs. 16%, p=0.001; 33% vs. 15%, p=0.001 and 25% vs. 7.3%, p=0.001, respectively). CONCLUSIONS: VTA occurred in 1.4% of pregnant women with cardiovascular disease, mainly in the third trimester, and was associated with heart failure during pregnancy. NYHA class before pregnancy was predictive. VTA during pregnancy had clear impact on fetal outcome.

Two cases of cardiac sarcoidosis in pregnant women with supraventricular arrhythmia.

We present two cases of cardiac sarcoidosis whose first presentation was in pregnancy. All findings confirmed the diagnosis of sarcoidosis with cardiac involvement in both patients. The first patient, a 37-year-old, presented with dizziness and atrial fibrillation at 16 weeks' gestation. Echocardiography revealed thickened interventricular septum with a speckled pattern. Cardiac MRI after delivery showed myocardial oedema/inflammation corresponding with the same regions with early enhancement and epicardial delayed enhancement in the basal to mid-inferoseptal and basal anterior left ventricular myocardial segments. Transbronchial biopsy revealed histology of scanty fragments of inflamed bronchial mucosa. The second patient, a 31-year-old, was 17 weeks pregnant when she presented with daily palpitations and shortness of breath. She had prolonged episodes of supraventricular tachycardia. Echocardiography revealed a speckled septal and right ventricular wall pattern. Cardiac MRI after delivery showed basal and mid-ventricular mesocardial and epicardial enhancement, most compatible with sarcoidosis.

Atrial Fibrillation or Flutter During Pregnancy in Patients With Structural Heart Disease: Data From the ROPAC (Registry on Pregnancy and Cardiac Disease).

OBJECTIVES: Atrial fibrillation (AF)/atrial flutter (AFL) during pregnancy in these women is associated with adverse outcome of pregnancy. BACKGROUND: The incidence, timing, and consequences of AF or AFL during pregnancy in patients with heart disease are not well known. METHODS: Between January 2008 to June 2011, 60 hospitals in 28 countries prospectively enrolled 1,321 pregnant women with congenital heart disease, valvular heart disease, ischemic heart disease, or cardiomyopathy in the ROPAC (Registry of Pregnancy and Cardiac Disease). We studied the incidence, onset, and predictors of AF/AFL during pregnancy and assessed the pregnancy outcome. An overview of the existing literature is provided. RESULTS: Seventeen women (1.3%) developed AF/AFL during pregnancy, mainly in the second trimester (61.5%). Univariable analysis identified the following pre-pregnancy risk factors for AF/AFL in pregnancy: AF/AFL before pregnancy (odds ratio [OR]: 7.1, 95% confidence interval [CI]: 1.5 to 32.8); mitral valvular heart disease (OR: 6.9, 95% CI: 2.6 to 18.3); beta-blocker use (OR: 3.3, 95% CI: 1.2 to 9.0); and left-sided lesions (OR: 2.9, 95% CI: 1.0 to 8.3). Maternal mortality was higher in women with than in women without AF/AFL (11.8% vs. 0.9%; p = 0.01), although heart failure was not seen more often. Low birth weight (<2,500 g) occurred more often in women with than in women without AF/AFL (35% vs. 14%; p = 0.02). CONCLUSIONS: AF/AFL occurs in 1.3% of pregnant patients with structural heart disease with a peak at the end of the second trimester. AF/AFL during pregnancy in cardiac patients is associated with unfavorable maternal outcome and also has an impact on fetal birth weight.