Scimitar syndrome in an infant with right lung hypoplasia, ventricular septal defect, and severe pulmonary hypertension.
Summary of "Scimitar syndrome in an infant with right lung hypoplasia, ventricular septal defect, and severe pulmonary hypertension."
A 5-month-old boy was presented for surgical repair of scimitar syndrome associated with right lung hyperplasia, severe pulmonary hypertension, ventricular septal defect (VSD), and atrial septal defect. The calculated shunt fraction (Qp/Qs) was 3.1:1.0, pulmonary vascular resistance was 4.6, and the perfusion lung scan showed a marked decrease (11%) in right pulmonary blood flow. Surgical repair was performed through the right fourth intercostal space with the patient supine. The anomalous vein was divided and interposed with a short azygos vein graft, followed by closure of the VSD. Finally, the interposed azygos vein was anastomosed to the left atrium. Although pulmonary artery pressure was normalized at the 2-year follow-up, cardiac catheterization 6 months after the operation demonstrated right pulmonary vein obstruction.
Department of Cardiac Surgery, Jikei University School of Medicine, 3-25-8 Nishishinbashi, Minato-ku, Tokyo, 105-8461, Japan, firstname.lastname@example.org.
This article was published in the following journal.
Name: General thoracic and cardiovascular surgery
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/20941566
- DOI: http://dx.doi.org/10.1007/s11748-009-0567-1
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Medical and Biotech [MESH] Definitions
An anomalous pulmonary venous return in which the right PULMONARY VEIN is not connected to the LEFT ATRIUM but to the INFERIOR VENA CAVA. Scimitar syndrome is named for the crescent- or Turkish sword-like shadow in the chest radiography and is often associated with hypoplasia of the right lung and right pulmonary artery, and dextroposition of the heart.
An autosomal dominant defect of cardiac conduction that is characterized by an abnormal ST-segment in leads V1-V3 on the ELECTROCARDIOGRAM resembling a right BUNDLE-BRANCH BLOCK; high risk of VENTRICULAR TACHYCARDIA; or VENTRICULAR FIBRILLATION; SYNCOPAL EPISODE; and possible sudden death. This syndrome is linked to mutations of gene encoding the cardiac SODIUM CHANNEL alpha subunit.
A chronic lung disease developed after OXYGEN INHALATION THERAPY or mechanical ventilation (VENTILATION, MECHANICAL) usually occurring in certain premature infants (INFANT, PREMATURE) or newborn infants with respiratory distress syndrome (RESPIRATORY DISTRESS SYNDROME, NEWBORN). Histologically, it is characterized by the unusual abnormalities of the bronchioles, such as METAPLASIA, decrease in alveolar number, and formation of CYSTS.
A malignant form of polymorphic ventricular tachycardia that is characterized by HEART RATE between 200 and 250 beats per minute, and QRS complexes with changing amplitude and twisting of the points. The term also describes the syndrome of tachycardia with prolonged ventricular repolarization, long QT intervals exceeding 500 milliseconds or BRADYCARDIA. Torsades de pointes may be self-limited or may progress to VENTRICULAR FIBRILLATION.
An oculomandibulofacial syndrome principally characterized by dyscephaly (usually brachycephaly), parrot nose, mandibular hypoplasia, proportionate nanism, hypotrichosis, bilateral congenital cataracts, and microphthalmia. (Dorland, 27th ed)