2013, Number 1
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Rev Cubana Pediatr 2013; 85 (1)
Echocardiographic morphology of the pulmonary atresia with intact ventricular septum. A 20-year study
González MAE, Serrano RG, Serrano SA, Savío BAF, Casanova ARI
Language: Spanish
References: 20
Page: 6-16
PDF size: 148.72 Kb.
ABSTRACT
Introduction: pulmonary atresia with intact ventricular system is a cardiovascular malformation accounting for 1% of congenital cardiopathies seen in the extrauterine life; due to its unfavorable outcomes, this disease is a true challenge for the contemporary medicine.
Objectives: the research study comprised the application of the classification guidelines, the characterization of the atresia outlet track, the morphological study of the right ventricle, the assessment of the tricuspid ring and the detection of anomalies in the coronary circulation.
Methods: forty three patients diagnosed with this disease and referred to "William Soler" pediatric cardiocenter from January 1992 to November 2011 were studied. Each case was performed bidimensional echocardiography and color-coding Doppler.
Results and conclusions: the study confirmed the predominance of the valvular morphological variant of the disease and the presence of moderate and severe levels of right ventricular hypoplexia associated with limited volumetric capacitance of this chamber, with tricuspid valvular hypodevelopment and with anomalous sinusoids-dependent coronary circulation. The permeable oral foramen was the interatrial septal defect that was most associated to this disease and several structural anomalies of the tricuspid system was evidenced in conjunction or not with the prevailing annular hypodevelopment.
REFERENCES
Shinebourne EA, Rigby ML, Carvalho JS. Pulmonary atresia with intact ventricular septum: from fetus to adult. Heart. 2008;94:1350-7.
Daubeney PE, Delany DJ, Anderson RH. Pulmonary atresia with intact ventricular septum: range of morphology in a population based study. J Am Coll Cardiol. 2002;39:1670-9.
Portela F, Marcos S. Atresia pulmonar con septo íntegro. Circ Cardiov. 2008;15(4):337-44.
Greenwold WE, DuShane JW, Burchell HB, Bruwer A, Edwards JE. Congenital pulmonary atresia with intact ventricular septum: two anatomic types. Circulation. 1956;14:945-6.
Daubeney PE, Wang D, Delany DJ. UK and Ireland collaborative study of pulmonary atresia with intact ventricular septum. Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study. J Thorac Cardiovasc Surg. 2005;130:1071-8.
Drant SE. The echocardiographic evaluation of pulmonary atresia with intact ventricular septum. Prog Pediatr Cardiol. 2001;13:165-75.
Yoshimura N, Yamaguchi M, Ohashi H. Pulmonary atresia with intact ventricular septum: strategy based on right ventricular morphology. J Thorac Cardiovasc Surg. 2003;126:1417-26.
Muñoz-Castellanos L, Galindo-Herrera M, Kuri-Nivon M. Hipoplasia ventricular derecha. Estudios morfométrico y morfológico. Archivos de Cardiología de México. 2007;77:181-93.
Salvin JW, McElhinney DW, Colan SD. Fetal tricuspid valve size and growth as predictors of outcome in pulmonary atresia with intact ventricular septum. Pediatrics. 2006;118:415-20.
Patel RG, Freedom RM, Moes CAF. Right ventricular volume determinations in 18 patients with pulmonary atresia and intact ventricular septum: analysis of factors influencing right ventricular growth. Circulation. 1980;61:428-40.
Burch TM, Mizuguchi KA, Wesley MC, Swanson TM. Echocardiographic Features of Pulmonary Atresia with Intact Ventricular Septum. International Anesthesia Research Society. 2008;107:1509-11.
DuBois D, DuBois EF. A formula to estimate the approximate surface area if height and weight be known. Arch Intern Medicine. 1916;17:863-71.
Lee YS, Kim YH, Hyum MC. Echocardiographic parameters of pulmonary atresia with intact ventricular septum. J Korean Pediatr Soc. 2003;46:484-9.
Schneider C, McCrindle BW, Carvalho JS. Development of Z-scores for fetal cardiac dimensions from echocardiography. Ultrasound Obstet Gynecol. 2005;26:599-605.
DeVore GR. The use of Z-scores in the analysis of fetal cardiac dimensions. Ultrasound Obstet Gynecol. 2005;26:596-8.
Satou GM, Perry SB, Gauvreau K, Geva T. Echocardiographic predictors of coronary artery pathology in pulmonary atresia with intact ventricular septum. Am J Cardiol. 2000;85:1319-24.
Zuberbuhler JR, Anderson RH. Morphological variations in pulmonary atresia with intact ventricular septum. Heart. 1979;41:281-8.
Gittenberger de Groot AC, Eralp I, Lie-Venema H, Bartelings MM, Poelmann RE. Development of the coronary vasculature and its implications for coronary abnormalities in general and specifically in pulmonary atresia without ventricular septal defect. Acta Paediatr Suppl. 2004;93:13-9.
Choi YH, Seo JW, Yun YS, Kim SH. Morphology of Tricuspid Valve in Pulmonary Atresia with Intact Ventricular Septum. Pediatr Cardiol. 1998;19:381-9.
Calder AL, Peebles CR, Occleshaw CJ. The prevalence of coronary arterial abnormalities in pulmonary atresia with intact ventricular septum and their influence on surgical results. Cardiol Young. 2007;17:387-96.