Pediatric pulmonary veno-occlusive disease associated with a novel BMPR2 variant.

Pulmonary veno-occlusive disease (PVOD) and idiopathic/heritable pulmonary arterial hypertension (I/HPAH) cause progressive PH on the distinct genetic impact. A 29-month-old boy presented with a loss of consciousness. He had severe PH refractory to pulmonary vasodilators. Hypoxemia and ground-glass opacity on the chest computed tomography were present, and significant pulmonary edema developed after the introduction of continuous intravenous prostaglandin I2 . Based on the clinical diagnosis of PVOD, he underwent a single living-donor lobar lung transplantation with the right lower lobe of his mother. The pathological findings of his explanted lung showed intimal thickening and luminal narrowing of the pulmonary vein. A genetic test revealed a novel heterozygous splice acceptor variant (c.77-2A>C) in BMPR2, which is typically associated with I/HPAH. This is the first pediatric case of PVOD with BMPR2 variant, supporting the concept that I/HPAH and PVOD are part of a spectrum of pulmonary vascular disease.

Transcatheter Closure of a Huge Ductus Arteriosus in a Severely Ill Neonate.

Hemodynamically significant patent ductus arteriosus (PDA) in preterm infants increases morbidity and mortality. Here we describe a 12-day-old neonate with a huge PDA who developed pulmonary hemorrhage following disseminated intravascular clotting and multiple organ failure. Medical treatment or surgical ligation could not be performed because of the patient's poor condition. Transcatheter closure using a commercially available device (Amplatzer Vascular Plug II) successfully treated the huge PDA without major complications. The Amplatzer Vascular Plug II approach might become a new option for PDA closure in small infants, including those who are critically ill.

Successful Treatment of Protein-Losing Enteropathy and Plastic Bronchitis by Biphasic Cuirass Ventilation in a Patient with Failing Fontan Circulation.

We present a 16-year-old male patient with hypoplastic left heart syndrome who developed protein-losing enteropathy (PLE) and plastic bronchitis (PB) after a Fontan operation. He received medical therapies, including albumin infusion, unfractionated heparin, and high-dose anti-aldosterone therapy but could not obtain clinical relief. Biphasic cuirass ventilation (BCV) led to expectoration of bronchial casts and prompt resolution of PB. Notably, clinical symptoms related to PLE were dramatically improved after starting BCV. A brief period of BCV increased stroke volume from 26±1.4 to 39±4.0 mL. This case suggests that BCV could be an effective treatment for PLE in patients with failing Fontan circulation.

Takotsubo cardiomyopathy associated with Paragonimiasis westermani.

An 11-year-old boy collapsed during morning assembly at his junior high school. The automated external defibrillator detected ventricular fibrillation and provided shock delivery. He was successfully resuscitated and reverted to sinus rhythm. Electrocardiography showed ST-T elevation in the precordial leads. Echocardiography and angiography demonstrated akinesia of the apex and mid-wall of the left ventricle with preserved contraction of the basal segments, which suggested Takotsubo cardiomyopathy. The patient and his family had often eaten uncooked crab, and his father had a past history of infection with Paragonimiasis westermani. The patient had had a persistent cough and chest pain for several weeks. Chest radiograph showed cystic cavities in the left upper lung. Microbiological examination of the sputum demonstrated an egg of P. westermani and immunological assay showed a raised antibody titre to P. westermani. On the12th day of admission, he developed seizures, and magnetic resonance imaging demonstrated cerebral involvement. After the administration of praziquantel for 3 days, the clinical manifestations improved immediately, and echocardiography normalised within 3 weeks. The patient was discharged on the 32nd day + and follow-up was normal. Takotsubo cardiomyopathy following a potentially fatal arrhythmia is a rare cardiac complication associated with pulmonary and central nervous system infection by P. westermani.

Pulmonary arterial resistance and compliance in preterm infants.

BACKGROUND: Preterm birth is known to be associated with an increased risk of pulmonary arterial hypertension, although how preterm birth influences pulmonary hemodynamics has not been fully understood. Pulmonary arterial resistance (Rp) and compliance (Cp) are important factors to assess the pulmonary circulation. The purpose of this study is to clarify the relationship between Rp and Cp in preterm infants. METHODS: We performed cardiac catheterization in 96 infants (50 males) with ventricular septal defect, and compared pulmonary hemodynamic parameters including Rp and Cp between preterm and full-term infants. RESULTS: Thirteen infants were preterm. There were no significant differences in sex, age, preoperative pulmonary arterial pressure, preoperative pulmonary-to-systemic flow ratio, and preoperative Rp between the 2 groups. However, preoperative Cp and resistor-capacitor (RC) time in preterm infants were significantly lower than those in full-term infants (2.1 vs 2.8mL/mmHg/m2 and 0.31 vs 0.36s, respectively; p<0.05 and p<0.01, respectively). Postoperative systolic and mean pulmonary arterial pressures were higher in preterm infants than those in full-term infants (29 vs 25mmHg and 18 vs 14mmHg, respectively; both p<0.01). It was also observed that postoperative Cp was lower in preterm infants, although postoperative Rp remained unchanged. CONCLUSIONS: We demonstrated that preterm infants with pulmonary arterial hypertension had lower Cp than full-term infants, causing a modest increase in pulmonary arterial pressure. It is important to consider the unique pulmonary vasculature characterized by lower Cp, when managing preterm infants with congenital heart disease.