Non-congenital heart disease related pulmonary arterial hypertension in 45 children / 中华实用儿科临床杂志

ABSTRACT

Objective To improve the knowledge of paediatric pulmonary arterial hypertension(PAH) and to elevate the level of early diagnosis and treatment.Methods The clinical data of 45 PAH patients admitted in Shanghai Children's Hospital from Jan.2006 to Dec.2012 were reviewed,including clinical manifestation,laboratory examination,diagnosis,treatment and prognosis.Results Of the 45 PAH patients,21 cases (46.7％) were male and 24 cases (53.3 ％) were female,with an average age of 2.5 years old.Among them idiopathic PAH was in 24 cases (53.3 ％) and secondary PAH was in 21 cases [including interstitial lung disease in 4 cases,upper airway obstruction in 3 cases,systemic lupus erythematosus in 3 cases,hepatic disease in 3 cases,including hepatic hemangioma 1 case,liver cirrhosis portal hypertension 1 case and autoimmune hepatic sarcoidosis 1 case,tachycardia induced cardiomyopathy in 2 cases,extensive pulmonary small artery stenosis in 2 cases,human immunodeficiency virus (HIV) infection in 1 case,hypothyroidism in 1 case,and familial PAH in 2 cases].Main clinical manifestations were anhelation after exercise (71.1％),fatigue (68.9％),cough (48.9％),chest tightness (26.7％),chest pain (33.3％),syncope (8.9％),et al.The most common physical signs were splitting of the second sound in pulmonary valve area (93.3％),followed by tricuspid murmur (77.8％),cyanosis (53.3％),hepatomegaly (42.2％),hydropericardium (28.9％),and oedema (11.1％),et al.Twenty-seven cases did cardiac catheterization,compared with idiopathic PAH and secondary PAH,pulmonary artery systolic pressure,mean pulmonary artery pressure,pulmonary capillary wedge pressure,pulmonary arteriolar resistance index had significant difference(P ＜ 0.05).Thirty-one cases' condition improved after treatment,11 cases without any improvement and 3 cases died during follow-up visit.Conclusions PAH is a rare disease with no specific symptom and can be easily misdiagnosed in children.Ultrasonic cardiogram and cardiac catheterization are helpful in diagnosis.Actively looking for the cause can improve the prognosis.