Image diagnosis: Eisenmenger’s syndrome in patients with simple congenital heart disease

Bu H, Gong X, Zhao T.

BMC Cardiovasc Disord. 2020 Apr 23;20(1):194. doi: 10.1186/s12872-020-01489-y.

PMID: 32326907 Free PMC Article

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Abstract

Background: Early identification of congenital heart disease (CHD) allows detection of the pulmonary arteriopathy in an early stage, and timely shunt closure can permanently reverse pulmonary arterial hypertension (PAH). However, surgical correction is not recommended in patients with irreversible PAH. Herein we report our experience about Eisenmenger’s syndrome in simple CHD.

Case presentation: From January 2017 to November 2018, a total of 8 CHD patients (3 ventricular septal defects (VSD), 2 atrial septal defects (ASD), and 3 patent ductus arteriosus (PDA), median age, 15.5 years [range, 3-18 years]) with PAH were detected by chest X-ray, electrocardiogram, transthoracic echocardiography (TTE), computed tomographic angiography (CTA) and cardiac catheterization. The median defect diameter, pulmonary artery pressure (PAP), pulmonary vascular resistance (PVR) were 16.5 mm (range, 3-30 mm), 75 mmHg (range, 60-86 mmHg), and 16 Woods units (range, 12-19 Woods units), respectively. Here, we report the representative cases of three types of simple CHD with irreversible PAH. The surgical correction was not performed in all patients who had fixed PAH and were referred to medical treatment.

Conclusions: PAH in CHD can be reversed by early shunt closure, but this potential is lost beyond a certain point of no return. This article highlights the essence of enhancing the level of healthcare and services in Chinese rural areas. Failure to accurately and timely assess PAH will delay effective treatment past optimal treatment time, and even lead to death.

 

Fig. 1 The representative image of PAH-ASD. a and b: Chest X-ray showed cardiomegaly and a bulging pulmonary artery at the upper left cardiac border; cde and f: Pulmonary arteriography showed reduced pulmonary capillary filling. PAH: pulmonary arterial hypertension; ASD: atrial septal defect; PA: pulmonary artery; RPA: right pulmonary artery; LPA: left pulmonary artery; RILA: right inferior lobe artery; LSLA: left superior lobe artery; LILA: left inferior lobe artery

Fig. 2 The representative image of PAH-VSD. a: Chest X-ray confirmed a bulging pulmonary artery at the upper left cardiac border; b: Right heart catheterization revealed a lager VSD; c and d: Pulmonary arteriography showed that the degree of pulmonary capillary filling decreased. PAH: pulmonary arterial hypertension; VSD: ventricular septal defect; PA: pulmonary artery; RPA: right pulmonary artery; LPA: left pulmonary artery; RILA: right inferior lobe artery

Fig. 3 The representative image of PAH-PDA. a: Chest X-ray showed a bulging pulmonary artery at the upper left cardiac border; b: Descending aortography revealed a large PDA; c and d: Pulmonary arteriography showed a large PDA and decreased pulmonary capillary filling.. PAH: pulmonary arterial hypertension; PDA: patent ductus arteriosus; PA: pulmonary artery; DAO: descending aorta; LILA: left inferior lobe artery

 

source:https://pubmed.ncbi.nlm.nih.gov/32326907/

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