Insufficient development of vessels and alveoli in lungs of infants with trisomy 18-Features of pulmonary histopathological findings from lung biopsy.

The aim of this study was to evaluate the features of pulmonary histopathological changes in cases of trisomy 18 complicated with congenital heart disease and pulmonary arterial hypertension. Twenty-eight patients with trisomy 18 underwent open lung biopsy at the time of primary operation in our hospital between 2008 and 2019. We compared these histopathological findings with those from previously described groups without trisomy 18.

In situ thrombosis of small pulmonary arteries in pulmonary hypertension developing after chemotherapy for malignancy.

A few reports have provided histopathological insight into pulmonary hypertension developing after antitumor chemotherapy. In general, plexogenic pulmonary arteriopathy is a commonly observed finding in patients with severe pulmonary hypertension. We herein report a novel pathological finding that may characterize the histopathological change occurring in patients with pulmonary hypertension after chemotherapy for malignancy.

An infant with primary pulmonary vein stenosis, associated with fatal occlusion of intraparenchymal small pulmonary veins.

Primary pulmonary vein stenosis (PVS) is rare within the pediatric population and its pathophysiology remains unclear, especially as to how the histopathology relates to its refractoriness to treatment. We report the case of a 4-month-old girl with primary PVS. The lesion in this patient was characterized by fatal obstruction of intraparenchymal small pulmonary veins, associated with localized stenosis at the four pulmonary veno-atrial junctions.

Medial defects of the small pulmonary arteries in fatal pulmonary hypertension in infants with trisomy 13 and trisomy 18.

Congestive heart failure is a major cause of early death in patients with trisomy 13 or 18 and congenital heart disease (CHD). Pulmonary artery banding for these patients early in life is preferred to protect the lungs from high pulmonary flow rates and improve survival. We performed open lung biopsies in 11 patients with trisomy 13 or 18 accompanied by CHD and severe pulmonary artery hypertension (PAH) between 2009 and 2011.

Histopathological examination by lung biopsy for the evaluation of operability and postoperative prognosis in patients with chronic thromboembolic pulmonary hypertension.

Background: To evaluate the prognosis after pulmonary thromboendarterectomy (PTE) in patients with chronic thromboembolic pulmonary hypertension (CTEPH), a lung biopsy was performed in 34 patients with central CTEPH and in 7 patients with peripheral CTEPH during PTE.

Methods And Results: Postoperative prognosis was classified from A to E based on the postoperative hemodynamic parameters and clinical condition, and was compared with the index of occlusion (IOCTEPH), which indicates the degree of occlusion in the small pulmonary arteries. Criteria of (A-E) were established only for central CTEPH.

Pulmonary vascular disease associated with pulmonary hypertension in 445 patients: diagnosis from lung biopsy and autopsy.

Purpose: Diagnosis from lung biopsy or autopsy was performed in 445 patients with congenital (385) or acquired (60) heart disease from all over Japan. The purpose of this study is the presentation of these prospective data collections.

Methods: Of the patients with congenital heart disease, 354 were biopsied to determine whether surgery was indicated.

Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association.

Background: Pulmonary arterial hypertension has been reported to be observed in association with acquired portal hypertension. However, the contribution of congenital anomalies occurring in the portal system to the development of pulmonary arterial hypertension remains to be elucidated.

Methods: Nine patients with congenital portosystemic venous shunt were studied from January 1990 through September 2005.

Two-stage procedure for pulmonary vascular obstructive disease in Down syndrome with congenital heart disease.

Background: Down syndrome patients are characterized by early progression of pulmonary vascular obstructive disease because of insufficient thickness of the pulmonary arterial media. For those with congenital heart disease (CHD) associated with pulmonary hypertension (PH), a 2-stage procedure of pulmonary artery banding (PAB) and then intracardiac repair (ICR) in early infancy is performed to prevent such pulmonary vascular diseases in early infancy.

Methods And Results: The subjects were 16 patients with Down syndrome who underwent lung biopsy during PAB and ICR.

Lung biopsy diagnosis of operative indication in secundum atrial septal defect with severe pulmonary vascular disease.

Objective: Surgical indication was determined by lung biopsy in 91 patients with secundum atrial septal defect (ASD) and severe pulmonary hypertension > 70 mm Hg of pulmonary arterial peak pressure and/or pulmonary vascular resistance of > 8 U/m(2).

Methods And Results: Pulmonary vascular disease (PVD) in ASD was classified into four types: (1) Musculoelastosis consisting of longitudinal muscle bundles and elastic fibers; surgery is indicated no matter how severely the peripheral small pulmonary arteries are occluded. Surgery was performed in all of the 20 patients, and the postoperative course was uneventful.

Reevaluation of histomorphometric analysis of lung tissue in decision making for better patient selection for fontan-type operations.

Background: The various types of cavopulmonary connection are occasionally unsuccessful even when the indications have been strictly fulfilled based on preoperative hemodynamic studies. We performed a detailed study of lung specimens from 60 patients who were judged to be candidates for the modified Fontan procedures based on a catheterizaton study in order to reevaluate the role of histologic studies of the pulmonary vasculature and to determine histologic criteria for the Fontan-type operation.

Methods: We performed a histomorphometric analysis of specimens from 53 biopsy and 7 autopsy cases (0.

Hypoplasia of the small pulmonary arteries in hypoplastic left heart syndrome with restrictive atrial septal defect.

Background: Restrictive atrial septal defect (ASD) (including intact atrial septum [IAS]) has been reported to be a risk factor that negatively impacts survival in hypoplastic left heart syndrome (HLHS). Although lymphangiectasia and "arterialization" of the veins of the lung in HLHS with restrictive ASD have been reported, they cannot fully explain the high mortality. We have introduced a new method of evaluating the development of the pulmonary vasculature in histological sections and used it to assess patients' lungs.

Atrial septal defect with borderline pulmonary vascular disease: surgery and long-term oral prostacyclin therapy for recalcitrant pulmonary hypertension.

The hemodynamic determination of operability in atrial septal defect (ASD) with severe pulmonary hypertension is problematic. Therefore, we perform an open lung biopsy prior to the corrective surgery in cases with pulmonary vascular resistance greater than 8 units x m2 and/or pulmonary arterial peak pressure greater than 70 mmHg. We present 4 cases showing occlusion of more than 70% of the small pulmonary arteries and arterioles by musculoelastosis, thromboembolism and mixed-type (musculoelastosis and plexogenic arteriopathy) which was considered borderline in terms of operability.

Hypoplasia of the small pulmonary arteries in total anomalous pulmonary venous connection with obstructed pulmonary venous drainage.

Objective: Preoperative pulmonary venous obstruction has been reported to be a risk factor negatively impacting survival in total anomalous pulmonary venous connection. We examined lung tissue from total anomalous pulmonary venous connection patients with pulmonary venous obstruction and demonstrated hypoplasia of small pulmonary arteries to elucidate the mechanism underlying the poor outcome.

Methods: Ten total anomalous pulmonary venous connection patients with preoperative pulmonary venous obstruction between the ages of 2 days and 10 months were studied.

Pathological lesions causing pulmonary hypertension after closure of a ventricular septal defect.

A 15-year-old boy with a ventricular septal defect, pulmonary hypertension, Down's syndrome, and extremely thickened media (ETM) of the small pulmonary arteries died of heart failure and pulmonary hypertension 13 years after intracardiac repair. Microscopic examination of lung specimens collected prior to the intracardiac repair and at the time of autopsy revealed that the ETM had remained unchanged and that the arteries connected to the vessels with ETM had become severely thickened. The present case shows that even a small percentage of arteries with ETM can cause pulmonary hypertension, and illustrates one of the mechanisms of how pulmonary hypertension can fail to be resolved after intracardiac repair.

Severe unilateral pulmonary vascular changes in child with polysplenia.

A 12-year-old boy with polysplenia and single ventricle experienced recurrent episodes of pneumonia, hemoptysis, and pulmonary hypertension. Unilateral pulmonary vein obstruction was diagnosed, and a left pneumonectomy was performed. Microscopy of the resected specimen revealed pulmonary veno-occlusive disease in the small pulmonary venules, and old arteritis in the small pulmonary arteries.