Peripartum outcomes in a large population of women with pulmonary arterial hypertension associated with congenital heart disease

2019 ◽  
Vol 26 (10) ◽  
pp. 1067-1076 ◽  
Author(s):  
Qiangqiang Li ◽  
Konstantinos Dimopoulos ◽  
Tianyang Liu ◽  
Zhuoyuan Xu ◽  
Qian Liu ◽  
...  
Keyword(s):  
Risk Factors ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Hypertensive Crisis ◽  
Eisenmenger Syndrome ◽  
Pulmonary Arterial ◽  
Pulmonary Hypertensive Crisis

Aims Pulmonary arterial hypertension is a severe complication in patients with congenital heart disease and poses a significant risk to women wishing to become pregnant. This study describes the clinical presentation, maternal outcomes and risk factors for the peripartum period in women with pulmonary arterial hypertension related to congenital heart disease (PAH-CHD). Methods All pregnant women with PAH-CHD who were admitted for delivery in a tertiary center between February 2011–September 2016 were included. Logistic regression analysis was used to identify predictors of the combined endpoint of maternal death, severe heart failure requiring treatment, or pulmonary hypertensive crisis. Results Ninety-three women (94 pregnancies) were included. Average age was 27.5 ± 4.4 years. Thirty (31.9%) patients had Eisenmenger syndrome, 51 (54.3%) had pulmonary arterial hypertension associated with systemic-to-pulmonary shunts, and 13 (13.8%) had pulmonary arterial hypertension with corrected congenital heart disease. Twenty-three (24.5%) women required admission for delivery within two days from presentation. Elective Cesarean section was performed in 95.7% of women, with intravertebral anesthesia in 93.6%. Fifty-one (54.2%) patients received pulmonary arterial hypertension therapies during pregnancy. Six (6.4%) women died, 33 (35.1%) developed heart failure and 10 (10.6%) had a pulmonary hypertensive crisis. Patients who met the combined endpoint ( n = 34, 36.2%) were more likely to have Eisenmenger syndrome or repaired defects ( p < 0.001). Other risk factors in the multivariate model included lower arterial blood oxygen saturation, higher brain natriuretic peptide, and pericardial effusion on echocardiography. Conclusion Maternal mortality and morbidity remain high in PAH-CHD patients, who should be counseled on the risks of pregnancy and managed in a tertiary multidisciplinary environment to improve prognosis.

Pulmonary Arterial Hypertension In Adults With Congenital Heart Disease: General Overview of Disease Mechanisms

2007 ◽  
Vol 6 (3) ◽  
pp. 121-125 ◽  
Author(s):  
Ingram Schulze-Neick ◽  
John E. Deanfield
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Vascular System ◽  
Hypertensive Crisis ◽  
Multidisciplinary Care ◽  
Structural Heart Disease ◽  
Pulmonary Arterial

Adults with congenital heart disease (CHD) have become a rapidly expanding group of complex patients requiring multidisciplinary care in specialty centers by those trained in CHD. They represent one of the most challenging subgroups of patients with pulmonary arterial hypertension (PAH) due to the presence of structural heart disease with or without coexisting cyanosis and its complications. The primary focus of attention for these patients is the lungs, whose vascular system is affected by shunt flow, or is also congenitally malformed, or has been altered by surgical procedures. When PAH develops, it affects physical exercise tolerance, travel to high altitudes, pregnancy, operability, and anesthesia (myocardial failure due to pulmonary hypertensive crisis), and thus general morbidity and mortality in this special patient group.

Abstract 14610: Renal Dysfunction in Adults With Congenital Heart Disease in Vietnam

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Ngoc Thanh Kim ◽  
Thanh Tung Le ◽  
Doan Loi Do ◽  
Thanh Huong Truong
Keyword(s):  
Risk Factors ◽  
Heart Failure ◽  
Atrial Fibrillation ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Renal Dysfunction ◽  
Congenital Heart ◽  
Pulmonary Arterial

Introduction: In Vietnam, knowledge about renal function in adults with congenital heart disease (CHD) is limited. Hypothesis: This study aims to estimate incidence of renal dysfunction in adults with congenital heart disease and risk factors. Methods: This is a cross-sectional study, including 365 CHD patients more than 16 years old. We collected clinical and para-clinical information, estimated glomerular filtration rate (GFR) and calculated the odds ratio (OR) for reduced GFR. Results: Totally, 52.8% patients had GFR < 90 ml/phút/1.73 m 2 . Logistic regression had confirmed the OR for GFR < 90 ml/phút/1.73 m 2 in the group > 60-years-old, the group with atrial fibrillation, the group with heart failure (based on NT-proBNP > 125 pmol/L), and the group with pulmonary arterial hypertension (based on pulmonary artery systolic pressure > 50 mmHg by echocardiography) were 6.46 (95% CI: 1.37 - 30.41), 7.58 (95% CI: 1.66 - 34.56), 2.98 (95% CI: 1.49 - 5.98) and 1.84 (95% CI: 1.02 - 3.33), respectively. Conclusions: Renal dysfunction is common in adults with CHD. Age > 60 years-old, atrial fibrillation, heart failure, and pulmonary arterial hypertension were risk factors for renal dysfunction in adults with CHD.

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