Hypoplastic Right Ventricle, Tricuspid Atresia, Double-outlet Left Ventricle with D-Transposition of the Great Arteries and Subaortic Ventricular Septal Defect; 2-D Echocardiographic Diagnosis

Year 2014, Volume: 17 Issue: 2, 145 - 147, 01.02.2013

Osman Yılmaz Ömer Kılıç Nilay Hakan

https://doi.org/10.4274/khj.4979

Abstract

Double outlet left ventricle is very rare congenital cardiac anomaly with numerous anatomical subtypes. Here, we presented and discussed a 2-day-old male baby with aorta and pulmonary artery arising from the left ventricle. Congenital heart disease anomalies observed in our patient included a well-developed pulmonary artery being posterior and to the left of the aorta, the aorta being anterior and to the right of the pulmonary artery (D-transposition of the great vessels), ostium secundum atrial septal defect, tricuspid artesia, severe hypoplastic right ventricle, subaortic ventricular septal defect, increased pulmonary artery blood flow with normal pulmonary artery pressure and mitral-pulmonary fibrous continuity.

Keywords

Double-outlet left ventricle, d- transposition of the great arteries, tricuspid atresia, hypoplastic right ventricle

Hipoplastik Sağ Ventrikül, Triküspit Atrezisi, Subaortik Ventriküler Septal Defekt ve Büyük Arterlerin D-Transpozisyonu Olan Çift Çıkışlı Sol Ventrikül; 2-D Ekokardiyografik Tanı

Abstract

Çift çıkışlı sol ventrikül, çeşitli anatomik tiplerinin olduğu çok nadir bir konjenital kardiyak anomalidir. Bu olgu sunumunda aorta ve pulmoner arteri sol ventrikülden çıkan iki günlük bir erkek bebek tartışıldı. Olgumuzda görülen konjenital kalp hastalığı anomalileri arasında; iyi gelişmiş bir pulmoner arterin arkadan ve soldan çıkması, aortanın önden ve sağdan çıkması (Büyük arterlerin d-transpozisyonu), ostium sekundum tipi atriyal septal defekt, triküspit atrezisi, şiddetli hipoplastik sağ ventrikül, subaortik ventriküler septal defekt, normal pulmoner arter basıncı ile birlikte pulmoner arter kan akımı artışı ve mitral - pulmoner fibröz devamlılığının görülmesi yer alıyordu.

Keywords

Çift çıkışlı sol ventrikül, büyük arterlerin d- transpozisyonu, triküspit atrezisi, hipoplastik sağ ventrikül

References

• Van Praagh R, Weinberg PM, Srebro JP. Double outlet left ventricle, In: Adams FH, Emmanouilides GL, Riemen-Schneider TA, eds. Heart Diseases in Infants Children and Adolescents. Baltimore: Williams and Wilkins, 1989:461-73.
• Sakakibara S, Takao A, Arai T, Hashimoto A, Nogi M. Both great vessels arising from the left ventricle (Double outlet left ventricle) (Origin of both great vessels from the left ventricle) Bull Heart Institute. Japan, 1967:66.
• Wilkinson JL. Double outlet left ventricle. In: Anderson RH, Macartney FJ, Shinebourne EA, Tynan M, eds. Paediatric cardiology, vol 2. Edinburgh: Churchill Living-Stone, 1987:889-911.
• Gouton M, Bozio A, Rey C, Sassolas F, Vaksmann G, Di Filippo S. (Double outlet left ventricle: a rare and unusual cardiopathy. Apropos of 7 new cases). Arch Mal Coeur Vaiss 1996;89:553-9.
• Imai-Compton C, Elmi M, Manlhiot C, Floh AA, Golding F, Williams WG, et al. Characteristics and outcomes of double outlet left ventricle. Congenit Heart Dis 2010;5:532-6.
• Menon SC, Hagler DJ. Double-outlet left ventricle: diagnosis and management. Curr Treat Options Cardiovasc Med 2008;10:448-52.
• Donald Jr H, William E. Double outlet left ventricle, In: Adams FH, Emmanouilides GL, Riemen-schneider TA, eds. Heart Diseases in Infants Children and Adolescents. 5th ed. Baltimore: Williams and Wilkins, 1995,1270-6.
• Marino B, Bevilacqua M. Double-outlet left ventricle: two-dimensional echocardiographic diagnosis. Am Heart J 1992;123:1075-7.
• Lopes LM, Rangel PI, Soraggi AM, Furlanetto BH, Furlanetto G. Double- outlet left ventricle. Echocardiographic diagnosis. Arq Bras Cardiol 2001;76:511-6.
• DeLeon SY, Ow EP, Chiemmongkoltip P, Vitullo DA, Quinones JA, Fisher EA, et al. Alternatives in biventricular repair of double-outlet left ventricle. Ann Thorac Surg 1995;60:213-6.
• Saylam GS, Sarıoğlu A. Functional single ventricle: management an therapeutic options.Turk Gogus Kalp Dama 1996;1:15-27.
• Freedom RM. The dinosaur and banding of the main pulmonary trunk in the heart with functionally one ventricle and transposition of the great arteries; a saga of evolution and caution. J Am Coll Cardiol 1987;10:427-9.
• Mayer JE Jr, Bridges ND, Lock JE, Hanley FL, Jonas RA, Castaneda AR. Factors associated with marked reduction in mortality for Fontan operations in patients with single ventricle. J Thorac Cardiovasc Surg 1992;103:444-51.