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1/88. Aberrant right pulmonary artery and double outlet ventricle: one-stage repair.

    We report a 13-month-old male child with anomalous origin of the right pulmonary artery from the ascending aorta and a double outlet right ventricle. Aortic wall was used for elongation of the pulmonary artery and side-to-end connection to the pulmonary trunk. Special emphasis is made on this particular operative technique for strictly laterally originating right pulmonary artery that requires no prosthetic material, avoids extreme stretching, and may enable normal growth potential. ( info)

2/88. double outlet right ventricle with anterior and left-sided aorta and subpulmonary ventricular septal defect.

    double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle. This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. We report the case of an 18-day-old infant with DORV and extremely rare anatomical features, such as anterior and left-sided aorta and subpulmonary ventricular septal defect (VSD). In addition to the anatomic features, the role of the echocardiogram for guiding the diagnosis and the surgical therapy of this congenital heart disease are discussed. ( info)

3/88. Extended septoplasty for subaortic stenosis developed 19 years after double-outlet right ventricle repair.

    A 27-year-old woman developed subaortic stenosis 19 years after double-outlet right ventricle repair. Subaortic stenosis was caused by a narrow ring of fibromuscular ridge associated with a bulge of the underlying septal muscle. The aortic valve was bicuspid and stenotic. We conducted extended septoplasty, replacing the aortic valve. Postoperative cineangiogram showed an adequate left ventricular outflow pathway. Double-outlet right ventricle repair may thus be followed by subaortic stenosis as long as 19 years after initial surgery. This lesion was assumed due to acquired disease secondary to flow disturbances in the left ventricular outflow, so reconstructing an adequate outflow pathway is effective and appears to help avoid recurring stenosis. ( info)

4/88. Omental flap for mediastinitis after median sternotomy in asplenia syndrome and gut malrotation.

    We describe a 12-year-old patient with asplenia syndrome and gut malrotation who, after an interim step before a modified Fontan operation, developed life-threatening mediastinitis. A flap of the omentum arising from the transverse colon, longitudinally located in the left of the abdomen, was created and transferred to the mediastinum after a division of the arterial arcade at its most caudal extent. Her postoperative course was uneventful, and 12 months later, the modified Fontan operation was successfully completed. Although visceral heterotaxy results in an omental deformation, it does not preclude the use of an omental flap. ( info)

5/88. An unusual case of double outlet right ventricle.

    A 17-year-old unmarried girl was admitted with the complaints of breathlessness on exertion, swelling of upper and lower limbs and intermittent fever for last 21 days. Past history revealed bluish discolouration of nails and tongue since birth along with recurrent dyspnoea. After thorough general and cardiovascular examination, a provisional diagnosis of tetralogy of fallot was made. Chest x-ray revealed cardiomegaly and absent main pulmonary artery segment. ECG revealed gross right axis deviation and right ventricular hypertrophy. Echocardiogram showed features of double outlet right ventricle. ( info)

6/88. A case of isotretinoin embryopathy with bilateral anotia and Taussig-Bing malformation.

    We report a newborn infant with multiple congenital anomalies (anotia and Taussig-Bing malformation) due to exposure to isotretinoin within the first trimester. In this paper we aim to draw to the fact that caution is needed when prescribing vitamin a-containing drugs to women of childbearing years. ( info)

7/88. The use of nonfluoroscopic catheter-based mapping system to perform radiofrequency ablation in complex ventricular tachycardia after cardiac surgery in congenital heart disease: a case report.

    A nonfluoroscopic electroanatomical cardiac mapping system (CARTO) integrates anatomical and electrophysiological information to reconstruct a three-dimensional activation map. Information from the CARTO system helps to reveal the mechanism and perform successful ablation in scar re-entry ventricular tachycardia after cardiac surgery. Three-dimensional activation and propagation mapping was performed in a patient with ventricular tachycardia after surgical correction of a double outlet right ventricle. The ventricular tachycardia appeared in two morphologies and were refractory to antiarrhythmic medication including amiodarone. Both ventricular tachycardias were re-entered using the ventriculotomy scar but rotated in different directions. Successful radiofrequency ablation was performed by creating a line of conduction block from the pulmonic valve to the ventriculotomy scar using entrainment mapping and the ablation lesion tagging technique. The CARTO system is useful in mapping and guiding the ablation of complex ventricular tachycardia after surgical correction in congenital heart disease ( info)

8/88. A thoracal spinal cord teratoma associated with Taussig-Bing anomaly in a newborn.

    BACKGROUND: Teratomas are the most common type of childhood germ cell tumor, arising in both ovary and extragonadal locations. From 40% to 50% of the extragonadal teratomas occur in the sacrococcygeal region. Teratomas in the head, neck, brain, mediastinum, abdominal, and spinal cord locations are seen less frequently. Congenital anomalies are observed up to 26% of the cases with teratoma. methods: A 2-day-old newborn was admitted to the hospital because of a mass in the interscapular region. She was evaluated for this and other anomalies. The mass was excised totally, and histopathologic evaluation was done. RESULTS: Histopathologic examination of the mass revealed a mature teratoma. In addition, the child had a Taussig-Bing anomaly of the heart. CONCLUSIONS: This appears to be the first report of the association between congenital thoracic spinal cord teratoma and Taussig-Bing anomaly, a specific type of transposition of the great arteries. ( info)

9/88. Surgical repair for double outlet right ventricle and intact ventricular septum.

    We report the case of a neonate with a complex cardiac anomaly that consisted of a double outlet right ventricle, intact ventricular septum, small left ventricle, and pulmonary stenosis who underwent surgical repair with a successful outcome. We have not previously found a case report of a patient with double outlet right ventricle and intact ventricular septum who is still alive and in good health at an intermediate postoperative follow-up. ( info)

10/88. Biventricular repair in a patient having double outlet right ventricle with unusual straddling of the mitral valve.

    The authors achieved biventricular repair in a 1-year-old boy having double outlet right ventricle and unusual straddling of the mitral valve. There was a substantial abnormal muscular bar present within the right ventricle, and some of the tendinous cords of the mitral valve were attached to this structure. Preoperative echocardiography was particularly informative in enabling the surgeon to arrange the optional operative procedures. The postoperative course was smooth and uneventful. ( info)
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