Normalization of Pulmonary Vascular Resistance Long After Successful Primary Sutureless Repair for Congenital Pulmonary Vein Stenosis.
Summary of "Normalization of Pulmonary Vascular Resistance Long After Successful Primary Sutureless Repair for Congenital Pulmonary Vein Stenosis."
A 3-month-old infant with perimembranous ventricular septal defect (VSD) and pulmonary hypertension presented at our center. Cardiac catheterization showed a systolic pulmonary-to-systemic arterial pressure ratio of 0.99 and a pulmonary vascular resistance of 9.32 μm(2). Pulmonary angiography showed focal orifice stenosis in the right lower and left lower and upper pulmonary veins, whereas the right upper pulmonary vein was atretic. Sutureless pericardial marsupialization concomitant with VSD patch closure was used to repair the three stenotic veins. Administration of home oxygen therapy and sildenafil citrate was continued after surgical repair. Postoperative catheterization 1.5 years after surgery showed patency of the three repaired veins and normalization of pulmonary vascular resistance.
Affiliation
Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, 5-7-1, Fujishirodai, Suita, Osaka, 565-8565, Japan, thoashi@surg1.med.osaka-u.ac.jp.
Journal Details
This article was published in the following journal.
Name: Pediatric cardiology
ISSN: 1432-1971
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/21509494
- DOI: http://dx.doi.org/10.1007/s00246-011-9999-3
Medical and Biotech [MESH] Definitions
Hypertension, Pulmonary
Increased VASCULAR RESISTANCE in the PULMONARY CIRCULATION, usually secondary to HEART DISEASES or LUNG DISEASES.
Capillary Resistance
The vascular resistance to the flow of BLOOD through the CAPILLARIES portions of the peripheral vascular bed.
Epoprostenol
A prostaglandin that is a powerful vasodilator and inhibits platelet aggregation. It is biosynthesized enzymatically from PROSTAGLANDIN ENDOPEROXIDES in human vascular tissue. The sodium salt has been also used to treat primary pulmonary hypertension (HYPERTENSION, PULMONARY).
Pulmonary Veno-occlusive Disease
Pathological process resulting in the fibrous obstruction of the small- and medium-sized PULMONARY VEINS and PULMONARY HYPERTENSION. Veno-occlusion can arise from fibrous proliferation of the VASCULAR INTIMA and VASCULAR MEDIA; THROMBOSIS; or a combination of both.
Pulmonary Heart Disease
Hypertrophy and dilation of the RIGHT VENTRICLE of the heart that is caused by PULMONARY HYPERTENSION. This condition is often associated with pulmonary parenchymal or vascular diseases, such as CHRONIC OBSTRUCTIVE PULMONARY DISEASE and PULMONARY EMBOLISM.
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