Form Of Scimitar Syndrome Research Articles

Pericardial tunnel technique in the surgical management of the vertical form of scimitar syndrome.

To optimize the surgical strategy and individualized treatment for scimitar syndrome (SS) by summarizing the clinical outcomes of the pericardial tunnel technique for the vertical form of SS at a single centre. The vertical form was defined as an angle of scimitar vein (SV) insertion to the inferior vena cava (θ) ≤ 45°, whereas the horizontal form was defined as θ > 45°. Nine patients with vertical form were operated on from June 2011 to June 2017. The mean age of patients during surgery was 3.1 ± 3.63 (range 0.3-12) years. Five patients were with infantile form and 4 with childhood/adult form per Dupuis' classification. The pericardial tunnel technique entailed attaching the SV to the right lateral pericardium and directing blood flow to the left atrium through a left atriotomy inside the pericardial tunnel. All patients had associated intracardiac anomalies repaired concomitantly; 3 patients had major collateral arteries occluded or ligated. There was no in-hospital postoperative death or residual SV obstruction. The postoperative ventilation time was 88.3 ± 63.8 (range 36-264) h. Eight survivors were followed up for 33.4 ± 19.2 (range 2-72) months, with 1 lost to follow-up. Echocardiography demonstrated that 7 survivors had no pulmonary hypertension and 1 had mild pulmonary hypertension; none developed SV obstruction during the follow-ups. The pericardial tunnel technique is suitable for the vertical form of SS and is a reasonable option for individualized treatment of SS.

Partial anomalous pulmonary venous connection (RCD code: II-3C.0)

A 48-year-old woman was admitted to our institution with a year’s history of uncharacteristic chest pain episodes and decrease in exercise tolerance. A CT lung scan performed nine months earlier revealed an anomalous vein arising from the right lower pulmonary lobe and draining into the inferior vena cava. A chest radiogram performed 2 months prior to the admission showed a curved shape adjacent to the right heart border. Right heart catheterization revealed: normal systolic, diastolic and mean pulmonary artery pressures, normal pulmonary resistance and insignificant shunt volume. Pulmonary artery angiography demonstrated an anastomotic vessel between the aberrant pulmonary vein and the right upper pulmonary vein. The patient was diagnosed with Partial Anomalous Pulmonary Venous Connection in form of Scimitar syndrome. Given a mild character of symptoms, no signs of the right-sided heart volume overload, insignificant shunt fraction and normal pulmonary circulation parameters, the patient was qualified to a conservative treatment with a close medical observation. JRCD 2013 ; 1 (2): 39–41 Key words: partial anomalous pulmonary venous connection, scimitar syndrome, left-to-right shunt, pulmonary hypertension

Endoscopic Correction of the Adult Form of Scimitar Syndrome and Mitral Regurgitation: Anatomic and Technical Considerations

Scimitar syndrome is a congenital cardiac anomaly characterized by anomalous venous drainage of the right lung into the inferior vena cava. We report the combination of scimitar syndrome and mitral regurgitation and describe port-access correction for the adult form by means of an intraatrial baffle combined with mitral valve repair. Related considerations and modifications required in the standard Heartport (Cardiovations, Somerville, NJ) technique are discussed.

Scimitar syndrome versus meandering pulmonary vein: evaluation with three-dimensional computed tomography

We report two cases of abnormal configuration of the pulmonary venous system of the right lung evaluated with the use of three-dimensional spiral computed tomography (3D-CT). In the first case, an unusual form of scimitar syndrome was detected, consisting of two scimitar veins uniting into a common trunk immediately before their confluence into the inferior vena cava (IVC). The second case concerns a meandering pulmonary vein that followed a circuitous course through the right lung and came in close proximity to the IVC, before draining the entire right lung to the left atrium. In both cases, 3D-CT enabled a non-invasive, detailed anatomic evaluation of the right pulmonary vascular bed.

Atypical infantile form of scimitar syndrome with bronchomalacia

A male infant presenting with severe heart failure and respiratory distress was diagnosed with a hypoplastic right lung, scimitar syndrome with pulmonary sequestration and multiple anomalous systemic arteries, left bronchomalacia, a large atrial septal defect and coarctation of the aorta. The infant underwent a successful combined treatment of surgical and transcatheter intervention, including coil embolization and endobronchial stenting.