Congenital aortic valve atresia is usually part of a developmental anomaly usually called
&#8216;hypoplastic left-heart syndrome&#8217;. To the best of our knowledge, most reported cases of aortic
atresia have been associated with hypoplasia of the ascending aorta, of the left ventricle, and of
the mitral valve. We report a patient presenting with aortic atresia with a ventricular septal
defect and a normally sized left ventricle. (Cardiol J 2011; 18, 3: 304&#8211;306

Esmaeilzadeh, Maryam

Naderi, Nasim

Tabaei, Ali Sadeghpour

English

Via Medica Journals

CASE REPORTCardiology Journal2011, Vol. 18, No. 3, pp. 304–306Copyright © 2011 Via MedicaISSN 1897–5593304 www.cardiologyjournal.orgAddress for correspondence:  Maryam Esmaeilzadeh, MD, FCAPSC, Shaheed Rajaei Cardiovascular Medical and ResearchCenter, P.O. Box: 1996911151, Vali-Asr Ave. Adjacent to Mellat Park, Tehran, Iran, tel: 00982123921, fax: 00982122055594,e-mail: meszadeh@rhc.ac.irReceived: 13.12.2009 Accepted: 29.12.2009Aortic atresia with normally developedleft ventricle in a young adultMaryam Esmaeilzadeh1, Nasim Naderi1, Ali Sadeghpour Tabaei21Department of Echocardiography, Shaheed Rajaei Cardiovascular Medical and Research Center, Tehran, Iran2Department of Cardiac Surgery, Shaheed Rajaei Cardiovascular Medical and Research Center, Tehran, IranAbstractCongenital aortic valve atresia is usually part of a developmental anomaly usually called‘hypoplastic left-heart syndrome’. To the best of our knowledge, most reported cases of aorticatresia have been associated with hypoplasia of the ascending aorta, of the left ventricle, and ofthe mitral valve. We report a patient presenting with aortic atresia with a ventricular septaldefect and a normally sized left ventricle. (Cardiol J 2011; 18, 3: 304–306)Key words: aortic atresia, left ventricle, echocardiographyIntroductionAortic atresia is usually associated with hypo-plasia of the left ventricle (LV) and mitral valve [1, 2].However 4–7% of patients with aortic atresia havea ventricular septal defect (VSD) at the same timewhich may allow for the development of a normalmitral apparatus and LV (Fig. 1) [3]. In these pa-tients, the VSD provides an ‘outlet’ for the deve-loping LV and mitral valve in utero. The mainte-nance of flow across the mitral valve and throughthe LV allows for their normal growth and deve-lopment [4]. Aortic atresia associated with an ade-quately developed LV and mitral valve has been re-cognized as a discrete anatomic-clinical entity [1,2]. It was first described by Lev [5] and subsequent-ly by Rosenquist et al. [6] in a case presenting withan aortopulmonary fenestration and interruptedaortic arch. This condition, which is relatively fa-vorable for surgery, accounts for only a small pro-portion of all cases of aortic atresia. Roberts et al.[7], in reviewing 73 necropsy cases, found threehearts with normal LV and mitral valves (4%). The samefrequency was reported by Freedom et al. [8] (sixFigure 1. Schematic diagram of a heart with aortic atre-sia, ventricular septal defect (VSD) and normally deve-loped left ventricle and mitral valve; AA — ascendingaorta;  DA — descending aorta; IVC — inferior venacava; SVC — superior vena cava; LS — left subclavianartery; LC — left carotid artery; RC — right coronaryartery; RS — right subclavian artery;  PDA — patentdactus arteriosus; LPA — left pulmonary artery; RPA —right pulmonary artery; LV — left ventricle; RV — rightventricle. Based on from [2].AASVCRPAPDA DALSLCRCRSLPAVSDLVRVIVC305Maryam Esmaeilzadeh et al., Aortic atresia with normally developed left ventriclewww.cardiologyjournal.orgcases [4%] out of 148), Thiene et al. [2] reporteda slightly higher percentage (four cases [7%] out of 58).Case reportThe patient was a 20 year-old man referred forechocardiography because of a history of mild fa-tigue and headache during exertion, but no dysp-nea. On physical examination, blood pressure andheart rate were normal; pulse oxymetry showed anoxygen saturation of 85% at room air. In cardiovas-cular examination, the point of maximal impulse wasdisplaced downwards and laterally; auscultationrevealed a normal S1 with mildly accentuated S2accompanied by a soft 3/6 systolic murmur in apex,left sterna border and base of heart. He had mildclubbing and no cyanosis. Other examinations wereunremarkable. The electrocardiogram showed nor-mal sinus rhythm, biatrial enlargement, extremeaxis deviation and right ventricular hypertrophy.Transthoracic and transesophageal echocardio-graphy showed situs solitus, D-loop, atrioventri-cular concordance, normal LV size and function,right ventricle enlargement and right ventricularhypertrophy, large outlet VSD (Fig. 2), absence ofaortic valve and very small ascending aorta dilatedpulmonary artery and its branches (Fig. 3), normalarch and branch vessels with retrograde flow fromdescending aorta, and a very large patent ductus ar-teriosus (Fig. 4). There was also a mild mitral re-gurgitation, moderate to severe tricuspid regurgi-tation, severe pulmonary hypertension (120 mm Hg),and small patent foramen oval.Figure 2. Transthoracic (top) and transesophageal (bot-tom) four-chamber views revealing very large ventricu-lar septal defect (VSD).Figure 4. Suprasternal long axis view shows large pa-tent ductus arteriosus (white arrow) and distal portionof aortic arch (yellow arrow). Color Doppler flow reve-als retrograde red flow through descending aorta.Figure 3. Parasternal short axis view shows dilated pul-monary artery (white arrow) and complete absence ofaortic valve (yellow arrow).306Cardiology Journal 2011, Vol. 18, No. 3www.cardiologyjournal.orgReferences1. Pellegrino PA, Thiene G. Aortic valve atresia with a normallydeveloped left ventricle. Chest, 1976; 69: 121–122.2. Thiene G, Gallucci V, Macartney FJ et al. Anatomy of aorticatresia, cases presenting with a ventricular septal defect. Circu-lation, 1979; 59: 1.3. Black MD, Smallhorn JF, Freedom RM et al. Aortic atresia witha ventricular septal defect: Modified single-stage neonatal biven-tricular repair. Ann Thorac Surg, 1999; 67: 751–755.4. Ohye RG, Kagisaki K, Lee LA, Mosca RS, Goldberg CS, Bove EL.Biventricular repair for aortic atresia or hypoplasia and ventricu-lar septal defect. J Thorac Cardiovasc Surg, 1999; 118: 648–654.5. Lev M. Pathologic anatomy and interrelationship of hypoplasiaof the aortic tract complexes. Lab Invest, 1952; 1: 61–70.6. Rosenquist GC, Taylor JFN, Stark J. Aortopulmonary fenestra-tion and aortic atresia. Report of an infant with ventricular septaldefect, persistent ductus arteriosus and interrupted aortic arch.Br Heart J, 1974; 36: 1146.7. Roberts WC, Perry LW, Chandra RS, Meyers GE, Shapiro SR,Scott LP. Aortic valve atresia: A new classification based onnecropsy study of 73 cases. Am J Cardiol, 1976; 37: 753.8. Freedom RM, Williams WG, Dische MR, Rowe RD. Anatomicalvariants in aortic atresia. Potential candidates for ventriculoaor-tic reconstitution. Br Heart J, 1976; 38: 821.9. Haworth SG, Reid L. Quantitative structural of pulmonary circu-lation in the newborn with the aortic atresia, stenosis or coarcta-tion. Thorax, 1977; 32: 121–128.DiscussionPalliative or corrective surgeryshould be per-formed in the neonatal period, before irreversiblechanges in the pulmonary circulation. An increasein the pulmonary arterial diameter and muscularitymay be apparent even within the first week of life [9].The presence of the normal LV and persistent pa-tency of ductus for early palliation pulmonary band-ing will be the required surgical procedure. Com-plete correction may be planned for a later stage.Our patient was a case of aortic atresia with nor-mally developed left ventricle. However, consider-ing systemic pulmonary hypertension (120 mm Hg)our patient was not a good candidate for surgicalcorrection and at this stage only heart and lungtransplantation should be considered. As with oth-er forms of Eisenmenger syndrome, our patient hashad a slowly progressive course with regard to thesymptoms and signs along with electrocardiogra-phic and hemodynamic data.AcknowledgementsThe authors do not report any conflict of inte-rest regarding this work.

Aortic atresia with normally developed left ventricle in a young adult

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Aortic atresia with normally developed left ventricle in a young adult

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