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A neonate with isolated combined aortic and pulmonary valvar stenosis
International Journal of Cardiology 116 (2007) e13 – e14 www.elsevier.com/locate/ijcard
Letter to the Editor
A neonate with isolated combined aortic and pulmonary valvar stenosis☆ Eduardo da Cruz a,b,c,⁎, Marie-Hélène Billieux c , Maurice Beghetti a a
Paediatric Cardiology Unit, Department of Paediatrics, Geneva Children's University Hospital, 6 rue Willy Donzé 1211-Geneva, Switzerland b Service of Paediatric and Neonatal Intensive Care, Department of Paediatrics, Geneva Children's University Hospital, 6 rue Willy Donzé 1211-Geneva, Switzerland c Foetal Unit, Division of Prenatal Medicine, Department of Gynaecology and Obstetrics, Geneva University Hospital, 6 rue Willy Donzé 1211-Geneva, Switzerland Received 17 July 2006; accepted 3 August 2006 Available online 7 November 2006
Abstract We present an extremely rare combination of isolated valvar pulmonary and aortic stenosis in a newborn patient. Most publications describe this feature in patients beyond the neonatal period and in association with other structural, myocardial or endocardial diseases. Our patient required an urgent and successful percutaneous pulmonary valvuloplasty due to poorly tolerated right ventricular hypertension and dilatation compressing the left ventricle. © 2006 Elsevier Ireland Ltd. All rights reserved. Keywords: Critical pulmonary stenosis; Aortic stenosis; Bicuspid aortic valve
T.B. was born by caesarean section at 41 weeks of term with a weight of 2.900 g. A prenatal ultrasound in the eve of his birth had documented a critical pulmonary valvular stenosis with a dilated, hypertrophic right ventricle (RV) and a ductal-dependent pulmonary blood flow. Immediately at birth, he was deeply cyanotic and an infusion of IV E1 prostaglandins was started. Post-natal echocardiography showed an astonishing combination of right- and left-heart obstruction. There were well developed and competent tricuspid and mitral valves and a moderately hypertrophic and dilated supra-systemic tripartite RV compressing the left ventricle (LV). The pulmonary ring was normal (8.5 mm) and the pulmonary valve was thickened, dysplastic and barely opened on dome (Fig. 1a). Peak systolic gradient between the RV and the pulmonary artery was 45 mm Hg with a largely ☆
The authors disclose any personal or financial support or involvement with organizations with financial interest in the matter. There isn't any conflict of interest. ⁎ Corresponding author. Foetal Unit, Division of Prenatal Medicine, Department of Gynaecology and Obstetrics, Geneva Children's University Hospital, 6, rue Willy Donzé, 1211-Geneva 14, Switzerland. Tel.: +41 22 382 4580; fax: +41 22 382 4546. E-mail address: [email protected] (E. da Cruz). 0167-5273/$ - see front matter © 2006 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2006.08.022
opened left-to-right ductus arteriosus (Fig. 1b). Pulmonary arteries were well developed with a post-stenotic dilatation of the trunk. The LV was normal. Inserted on a normal ring, the aortic valve was bicuspid, thickened, dysplastic and had a restricted systolic motion (Fig. 1c,d) the LV to aorta peak gradient being of 36 mm Hg. Coronary arteries had a normal distribution but looked globally dilated. Cardiac catheterization confirmed the echocardiographic diagnosis. There was a marked supra-systemic RV pressure. Coronary-to-RV fistulae were ruled-out. The pulmonary valve was dilated percutaneously with 8 and 10 mm Tyshak Numed® balloons with an excellent immediate result (RV pressure dropped to infra-systemic levels and the RV-PA gradient decreased from 55 of 10 mm Hg). Subsequent progression was uneventful, prostaglandins were withdrawn 24 h later and the patient remained slightly cyanotic (80% of saturation) secondarily to a right-to-left shunt through the foramen ovale. Residual pulmonary valvular systolic gradient remained at 8 mm Hg with a mild pulmonary regurgitation and the aortic gradient decreased to 16 mm Hg at the closure of the ductus arteriosus. The extraordinary combination of isolated valvar aortic and pulmonary stenosis is extremely rare and has been seldom reported without any other structural cardiac
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E. da Cruz et al. / International Journal of Cardiology 116 (2007) e13–e14
Fig. 1. a. Right-sided obstruction: echocardiography showing a thickened and dysplastic pulmonary valve (arrow). b. Right-sided obstruction: a largely opened left-to-right shunt through the ductus arteriosus (⁎). c. and d. Left-sided obstruction: echocardiography documenting a stenotic bicuspid aortic valve (arrow) in both parasternal long-axis (c) and short-axis (d) views (⁎).
abnormality [1–5]. In our case, the right sided obstruction was markedly pronounced when compared to the left obstruction and poorly tolerated justifying the pulmonary valvuloplasty. We hypothetized that the degree of left obstruction was conditioned by the massive venous return to the left atrium due to a large left-to-right shunt through the ductus arteriosus and this was confirmed with the significant decrease of the aortic valvar systolic gradient when the ductus arteriosus closed. Acknowledgement The authors would like to acknowledge Mrs. MarieHélène Decruy, senior echocardiographist for her essential commitment to our patients.
References [1] Spaetgens RL, Duncan WJ, Taylor GP. Supravalvar aortic stenosis and peripheral vascular stenoses. Cardiol Young 2002;12:290–3. [2] Milazzo AS, Golioto A, Camitta MGW, et al. An infant with subvalvar aortic stenosis, subvalvar and valvar pulmonary stenosis, severe biventricular hypertrophy and pulmonary hemorrhage. Pediatr Cardiol 2003;24:169–71. [3] Beard EF, Cooley DA, Latson JR. Combined congenital subaortic stenosis and infundibular subpulmonic stenosis. Arch Intern Med 1957;100:647–50. [4] Nadas AS, Van der Hauwaert L, Hauck AJ, et al. Combined aortic and pulmonic stenosis. Circulation 1962;5:346–55. [5] Shemin RJ, Kent KM, Roberts WC. Syndrome of valvular pulmonary stenosis and valvular aortic stenosis with atrial septal defect. Br Heart J 1979;42:442–6.
Letter to the Editor
A neonate with isolated combined aortic and pulmonary valvar stenosis☆ Eduardo da Cruz a,b,c,⁎, Marie-Hélène Billieux c , Maurice Beghetti a a
Paediatric Cardiology Unit, Department of Paediatrics, Geneva Children's University Hospital, 6 rue Willy Donzé 1211-Geneva, Switzerland b Service of Paediatric and Neonatal Intensive Care, Department of Paediatrics, Geneva Children's University Hospital, 6 rue Willy Donzé 1211-Geneva, Switzerland c Foetal Unit, Division of Prenatal Medicine, Department of Gynaecology and Obstetrics, Geneva University Hospital, 6 rue Willy Donzé 1211-Geneva, Switzerland Received 17 July 2006; accepted 3 August 2006 Available online 7 November 2006
Abstract We present an extremely rare combination of isolated valvar pulmonary and aortic stenosis in a newborn patient. Most publications describe this feature in patients beyond the neonatal period and in association with other structural, myocardial or endocardial diseases. Our patient required an urgent and successful percutaneous pulmonary valvuloplasty due to poorly tolerated right ventricular hypertension and dilatation compressing the left ventricle. © 2006 Elsevier Ireland Ltd. All rights reserved. Keywords: Critical pulmonary stenosis; Aortic stenosis; Bicuspid aortic valve
T.B. was born by caesarean section at 41 weeks of term with a weight of 2.900 g. A prenatal ultrasound in the eve of his birth had documented a critical pulmonary valvular stenosis with a dilated, hypertrophic right ventricle (RV) and a ductal-dependent pulmonary blood flow. Immediately at birth, he was deeply cyanotic and an infusion of IV E1 prostaglandins was started. Post-natal echocardiography showed an astonishing combination of right- and left-heart obstruction. There were well developed and competent tricuspid and mitral valves and a moderately hypertrophic and dilated supra-systemic tripartite RV compressing the left ventricle (LV). The pulmonary ring was normal (8.5 mm) and the pulmonary valve was thickened, dysplastic and barely opened on dome (Fig. 1a). Peak systolic gradient between the RV and the pulmonary artery was 45 mm Hg with a largely ☆
The authors disclose any personal or financial support or involvement with organizations with financial interest in the matter. There isn't any conflict of interest. ⁎ Corresponding author. Foetal Unit, Division of Prenatal Medicine, Department of Gynaecology and Obstetrics, Geneva Children's University Hospital, 6, rue Willy Donzé, 1211-Geneva 14, Switzerland. Tel.: +41 22 382 4580; fax: +41 22 382 4546. E-mail address: [email protected] (E. da Cruz). 0167-5273/$ - see front matter © 2006 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2006.08.022
opened left-to-right ductus arteriosus (Fig. 1b). Pulmonary arteries were well developed with a post-stenotic dilatation of the trunk. The LV was normal. Inserted on a normal ring, the aortic valve was bicuspid, thickened, dysplastic and had a restricted systolic motion (Fig. 1c,d) the LV to aorta peak gradient being of 36 mm Hg. Coronary arteries had a normal distribution but looked globally dilated. Cardiac catheterization confirmed the echocardiographic diagnosis. There was a marked supra-systemic RV pressure. Coronary-to-RV fistulae were ruled-out. The pulmonary valve was dilated percutaneously with 8 and 10 mm Tyshak Numed® balloons with an excellent immediate result (RV pressure dropped to infra-systemic levels and the RV-PA gradient decreased from 55 of 10 mm Hg). Subsequent progression was uneventful, prostaglandins were withdrawn 24 h later and the patient remained slightly cyanotic (80% of saturation) secondarily to a right-to-left shunt through the foramen ovale. Residual pulmonary valvular systolic gradient remained at 8 mm Hg with a mild pulmonary regurgitation and the aortic gradient decreased to 16 mm Hg at the closure of the ductus arteriosus. The extraordinary combination of isolated valvar aortic and pulmonary stenosis is extremely rare and has been seldom reported without any other structural cardiac
e14
E. da Cruz et al. / International Journal of Cardiology 116 (2007) e13–e14
Fig. 1. a. Right-sided obstruction: echocardiography showing a thickened and dysplastic pulmonary valve (arrow). b. Right-sided obstruction: a largely opened left-to-right shunt through the ductus arteriosus (⁎). c. and d. Left-sided obstruction: echocardiography documenting a stenotic bicuspid aortic valve (arrow) in both parasternal long-axis (c) and short-axis (d) views (⁎).
abnormality [1–5]. In our case, the right sided obstruction was markedly pronounced when compared to the left obstruction and poorly tolerated justifying the pulmonary valvuloplasty. We hypothetized that the degree of left obstruction was conditioned by the massive venous return to the left atrium due to a large left-to-right shunt through the ductus arteriosus and this was confirmed with the significant decrease of the aortic valvar systolic gradient when the ductus arteriosus closed. Acknowledgement The authors would like to acknowledge Mrs. MarieHélène Decruy, senior echocardiographist for her essential commitment to our patients.
References [1] Spaetgens RL, Duncan WJ, Taylor GP. Supravalvar aortic stenosis and peripheral vascular stenoses. Cardiol Young 2002;12:290–3. [2] Milazzo AS, Golioto A, Camitta MGW, et al. An infant with subvalvar aortic stenosis, subvalvar and valvar pulmonary stenosis, severe biventricular hypertrophy and pulmonary hemorrhage. Pediatr Cardiol 2003;24:169–71. [3] Beard EF, Cooley DA, Latson JR. Combined congenital subaortic stenosis and infundibular subpulmonic stenosis. Arch Intern Med 1957;100:647–50. [4] Nadas AS, Van der Hauwaert L, Hauck AJ, et al. Combined aortic and pulmonic stenosis. Circulation 1962;5:346–55. [5] Shemin RJ, Kent KM, Roberts WC. Syndrome of valvular pulmonary stenosis and valvular aortic stenosis with atrial septal defect. Br Heart J 1979;42:442–6.