Left Hemitruncus With Tetralogy of Fallot and Right Aortic Arch: Rare Survival Beyond the First Decade Sudhanshu Kumar Dwivedi, Sudarshan Kumar Vijay, Sharad Chandra & Ram Kirti Saran Pediatric Cardiology ISSN 0172-0643 Pediatr Cardiol DOI 10.1007/s00246-012-0291-y
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Author's personal copy Pediatr Cardiol DOI 10.1007/s00246-012-0291-y
IMAGES IN PEDIATRIC CARDIOLOGY
Left Hemitruncus With Tetralogy of Fallot and Right Aortic Arch: Rare Survival Beyond the First Decade Sudhanshu Kumar Dwivedi • Sudarshan Kumar Vijay Sharad Chandra • Ram Kirti Saran
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Received: 24 December 2011 / Accepted: 28 February 2012 Ó Springer Science+Business Media, LLC 2012
Abstract Hemitruncus is an uncommon congenital anomaly that has been described in isolation or in association with other congenital cardiac malformations. This report describes a rare case of left hemitruncus with tetralogy of Fallot and a rightsided aortic arch. The patient presented to us in the early second decade. The diagnosis was suspected with echocardiography and confirmed with cardiac catheterization. The patient underwent successful surgical correction of the anomaly. Keywords
Aortic arch Hemitruncus Tetralogy of Fallot
Case Report A 12-year-old boy was admitted to our department with history of exertional dyspnoea (New York Heart Association [NYHA] class 2) and cyanosis since early childhood. Physical examination exhibited central cyanosis, clubbing, a pulse rate of 80 bpm, blood pressure of 116/70 mmHg, and a resting systemic oxygen saturation of 82 % with room air. At precordial palpation, right ventricular hyperdynamic impulse was felt, and a grade 3/6 ejection systolic murmur was heard over the pulmonary area. The remainder of the systemic examination was unremarkable. The patient had a hemoglobin level of 18.5 gm/dl and a hematocrit level of 58 %. An electrocardiogram showed normal sinus rhythm, right axis deviation, and right Electronic supplementary material The online version of this article (doi:10.1007/s00246-012-0291-y) contains supplementary material, which is available to authorized users. S. K. Dwivedi S. K. Vijay (&) S. Chandra R. K. Saran Department of Cardiology, Chatrapati Shahuji Maharaj Medical University (King George Medical College), Chowk, Lucknow 226003, UP, India e-mail:
[email protected]
ventricular hypertrophy. Chest X-ray with a posteroanterior view showed moderate cardiomegaly, with prominence of vascular markings in the left lung field and decreased pulmonary vasculature in the right lung (Fig. 1). A two-dimensional transthoracic echocardiogram showed a large perimembranous ventriculoseptal defect (VSD) (1.2 cm) with overriding of the aorta (Fig. 2a). The parasternal short-axis view disclosed a 98-mmHg gradient at the right ventricular infundibular level, with nonvisualization of the left pulmonary artery (Fig. 2b). Cardiac catheterization confirmed the presence of a large VSD, overriding of the aorta and equal right and left ventricular systolic pressures. A right ventriculogram with a pulmonary arteriography showed the right pulmonary artery as the sole branch of the main pulmonary trunk and right ventricular infundibular obstruction (Fig. 3a). Aortography exhibited the left pulmonary artery arising from the ascending aorta, with the presence of a right-sided aortic arch (Fig. 3b). The left pulmonary vascular bed was reactive at testing with nitroglycerin as left pulmonary artery systolic pressure decreased significantly. The pulmonary vascular resistance decreased from 8 to 6 Wood units after inhaled nitric oxide. The patient underwent fenestrated patch repair of the VSD, resection of the infundibular stenosis with dissection, and end-to-side anastomosis of the left and main pulmonary arteries. At this writing, the boy is doing well after a 6-month follow-up period, with improved functional capacity and no systemic desaturation.
Discussion The anomalous origin of a pulmonary artery from the aorta is described as hemitruncus. Right hemitruncus is more
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Fig. 1 Chest X-ray. Posterior anterior view showing differential lung vascularity with increased vascular prominence (white arrows) on the left side and relative oligemic lung fields on the right side
Fig. 2 a Two-dimensional transthoracic echocardiogram (parasternal long-axis view) showing a large subaortic ventriculoseptal defect (VSD) with overriding of the aorta. b Parasternal short-axis view Fig. 3 a Right ventriculography with pulmonary angiogram showing infundibular pulmonary stenosis (PS) and the main pulmonary trunk continuing as the right pulmonary artery (RPA). b Ascending aortogram showing the origin of the left pulmonary artery (LPA) from the ascending aorta and the right-sided aortic arch (AA). RV right ventricle
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common than left hemitruncus and usually is described in isolation [2]. Tetralogy of Fallot is rarely associated with hemitruncus, and in one large series, its incidence was found to be 0.4 % [1]. Left hemitruncus is seen much more often with tetralogy of Fallot than right hemitruncus [6]. Other congenital defects associated with hemitruncus include aortopulmonary window, coarctation of the aorta, and type A interrupted aortic arch. Anomalous origin of the left pulmonary artery is thought to result from failure of development of the left sixth arch and persistence of the left fifth arch [2]. Early detection and repair of this anomaly is important because most of the children do not survive due to early development of pulmonary vascular obstructive disease if it is left untreated [5]. To the best of our knowledge, the literature describes only four cases [3, 4, 6] of left hemitruncus and tetralogy of Fallot with survival beyond the first decade.
showing a high-velocity jet at the right ventricle infundibular level and nonvisualization of the left pulmonary artery
Author's personal copy Pediatr Cardiol Acknowledgments There has been no financial assistance and conflict of interest among the authors.
References 1. Changela V, John C, Maheswari S (2010) Unusual cardiac associations with tetralogy of Fallot: a descriptive study. Pediatr Cardiol 31:785–791 2. Diab K, Richardson R, Pophal S, Alboliras E (2010) Left hemitruncus associated with tetralogy of Fallot: fetal diagnosis and postnatal echocardiographic and cardiac computed tomographic confirmation. Pediatr Cardiol 31:534–537
3. Kutsche LM, Van Mierop LHS (1988) Anomalous origin of a pulmonary artery from the ascending aorta: associated anomalies and the pathogenesis. Am J Cardiol 61:850–856 4. Mittal PK, Agarwal SK, Ghosh PK (1993) Isolated anomalous origin of left pulmonary artery from the ascending aorta in an adult. J Thorac Cardiovasc Surg 106:1220–1223 5. Peng EW, Shanmugam G, Macarthur KJ, Pollock JC (2004) Ascending aortic origin of a branch pulmonary artery: surgical management and long-term outcome. Eur J Cardiothorac Surg 26: 762–766 6. Soylu M, Demir AD, Tikiz H, Kisacik H, Korkmaz S (2000) Left hemitruncus associated with tetralogy of Fallot: a case report. Catheter Cardiovasc Interv 51:58–60
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