Since the first description of application of transcatheter methods to treat pulmonary and tricuspid stenoses by Rubio-Alvarez and Limon-Lason in early 1950s,1 a number of other transcatheter methods to treat congenital heart defects have been described, reviewed elsewhere.2 Most commonly, the trans-catheter methods address single defects. More recently, however, concurrent transcatheter therapy of two or more lesions has been undertaken.3–18 The objectives of this presentation are to present a child who underwent successful concurrent trans-catheter treatment of a rare combination of severe aortic stenosis and moderate-sized patent ductus arteriosus (PDA), discuss technical details of concurrent treatment of both these lesions by transcatheter methodology and to review utility of trans-catheter techniques in managing two or more cardiac defects simultaneously in the catheterization laboratory.
Case Report. We present a 1.5-year-old girl who was referred to us with history of PDA and aortic stenosis (AS) for further evaluation and management. A murmur was initially detected at the age of two weeks and an echocardiogram done at that time showed bicuspid aortic valve with a peak instantaneous gradient of 32 mmHg, a small PDA and a small mid-muscular ventricular septal defect, the latter two with left-to-right shunt. She was locally followed periodically, but when she developed easy fatigability and tachypnea and failed to thrive, she was referred to us for consideration of transcatheter treatment. On examination, the weight was 9.6 kg (at 5th percentile), upper and lower extremity blood pressures were normal, the first and second heart sounds were normal with an ejection systolic click best heard at the apex, and a grade 3/6 ejection systolic murmur best heard at the upper right sternal border with radiation to carotid vessels. Echocardiogram showed a moderate-sized PDA with left-to-right shunt. No ventricular septal defect was seen, and presumably closed spontaneously. The aortic valve appeared bicuspid with a Doppler peak instantaneous gradient of 70 mmHg and a mean gradient of 40 mmHg.
Cardiac catheterization was performed via the right femoral vein and artery. Heparin was administered and activated clotting times were monitored. Systemic venous oxygen saturations were normal in the vena cavae, right atrium and right ventricle. There was moderate step-up in saturations in the main and branch pulmonary arteries as a result of shunting across the PDA. The right ventricular (50/10 mmHg) and pulmonary artery pressures (43/29 mmHg) were mildly elevated, with no significant gradient across the pulmonary valve. Left ventricular pressure was 126/6 mmHg and the ascending and descending aortic pressures were 62/38 mmHg, respectively. The peak-to-peak systolic gradient across the aortic valve was 64 mmHg and there was no gradient across the aortic arch. The cardiac index was normal and the pulmonary index was increased, giving a calculated Qp:Qs of 2.22:1. Pulmonary vascular resistance (1.3 Wood units) was within normal limits. Aortic arch cineangiogram showed Krichenko Type A-1 PDA (Figure 1A) with good opacification of the main and branch pulmonary arteries.
A 4 French (Fr) multipurpose catheter (Cordis, Miami, Florida) was used to cross the PDA from the pulmonary artery into the descending aorta and was replaced with a 6 Fr Amplatzer delivery sheath (AGA Medical, Golden Valley, Minnesota) over an exchange-length extra-stiff Amplatz guidewire (Cook, Bloomington, Indiana). Based on the minimal ductal diameter of 3 mm, an 8/6 Amplatzer Duct Occluder (AGA Medical) was selected and implanted across the PDA (Figure 1B). However, the device was not released. Left ventricular test angiography revealed good position of the device without any obstruction of the descending aorta. There was no residual shunt across the device.
Left ventricular systolic pressure did not change following effective occlusion of the ductus. Based on the echocardiographic and angiographic aortic valve annular measurements (12–13 mm), a 12 mm diameter, 3 cm long Tyshak-II balloon catheter (NuMed Inc.) was selected for valvuloplasty.19 Three balloon inflations at 3–3.5 atm pressure were performed; the balloon waisting was abolished (Figures 2A and 2B). After valvuloplasty, the peak-to-peak aortic valve gradient decreased from 64 mmHg to 19 mmHg. An aortic root cineangiogram did not show any aortic insufficiency. Measurements of the pressures on pullback revealed no gradient across the aortic arch. At the conclusion of the procedure, the right ventricular, pulmonary artery and aortic oxygen saturations did not reveal any residual shunt, but the pulmonary artery pressures decreased toward normal (Table 1).
Clinical follow-up at 1, 6 and 18 months after the procedure revealed an asymptomatic child with progressive weight gain (weights between 25th and 50th percentile). There was a grade 1–2/6 ejection systolic murmur at the right upper sternal border and no diastolic murmur was heard. Follow-up echocardiograms on 1 day and 1, 6 and 18 months following the procedure revealed minimal residual gradients, trivial to no aortic insufficiency and no residual shunt across the device (Table 1).
Discussion. Whereas treatment of single defects by transcatheter methodology as an alternative to surgery is an accepted mode of management for several defects, multiple defects have generally been treated with surgical intervention. However, over the last decade, a number of cardiologists3–18 have treated multiple defects by transcatheter intervention (Table 2); the most common defects so treated are pulmonary stenosis and atrial septal defect. In general, the first intervention is performed taking into consideration that this is not disturbed by the second intervention. The preferred approach seems to be guided by the principle of "easy first, hard second," as was done in our patient.
The case presented in this report illustrates the feasibility of concurrent transcatheter intervention to successfully treat aortic stenosis and PDA. We initially occluded the ductus with an Amplatzer device, but did not release the device to prevent inadvertent dislodgement of the device during catheter manipulation for balloon aortic valvuloplasty. This also allowed us to ensure that the severity of aortic valve stenosis was not related to shunt across the PDA since it has been reported that some of the aortic valve gradient may be related to ductal shunt.20 In our case, the left ventricular pressure did not change following ductal occlusion. Therefore, we went ahead with balloon aortic valvuloplasty. Excellent immediate and follow-up results with relief of symptoms, improvement in weight percentile, complete closure of PDA and minimal residual aortic gradient were achieved in our case. Concurrent percutaneous management of AS and PDA has been undertaken by other workers in the past;17 Atiq et al treated three children with PDA and AS, two with severe left ventricular dysfunction, with resultant complete closure of the PDA in 75% patients and reduction of valve gradient by 70%. The ventricular function in both children who had diminished function prior to intervention improved. Based on our experience and that of others,17 we would recommend concurrent catheter intervention for patients with PDA and moderate-to-severe AS.
- Rubio-Alvarez V, Limon-Lason R, Soni J. Valvulotomias intracardiacas por medio de un cateter. Arch Inst Cordiol Mexico 1952;23:183.
- Rao PS. Interventional pediatric cardiology: State of the art and future directions. Pediat Cardiol 1998;19:107–124.
- Ing FF, McMahon WS, Johnson GL, et al. Single therapeutic catheterization to treat coexisting coarctation of the aorta and patent ductus arteriosus. Am J Cardiol 1997;79:535–537.
- Yip WC, Chan KY, Godman MJ. Simultaneous transcatheter valvuloplasty and Amplatzer septal occlusion for pulmonary valvar stenosis and secundum atrial septal defect. Ann Acad Med Singapore 1998;27:578–581.
- Hakim F, Hawelleh AA, Goussous Y, Hijazi ZM. Simultaneous stent implantation for coarctation of the aorta and closure of patent ductus arteriosus using the Amplatzer duct occluder. Catheter Cardiovasc Interv 1999;47:36–38.
- Wahl A, Windecker S, Misteli M, Meier B. Combined percutaneous pulmonary valvuloplasty and atrial septal defect closure for pulmonary valvular stenosis and associated secundum atrial septal defect in an adult. Catheter Cardiovasc Interv 2001;53:68–70.
- Liang CD, Ko SF, Tiao MM. False aneurysm and mediastinal hematoma: Complications of simultaneous transcatheter therapy for coarctation of the aorta and patent ductus arteriosus in an infant. J Invasive Cardiol 2001;13:710–712.
- Shrivastava S, Radhakrishnan S. Concurrent percutaneous atrial septal defect closure and pulmonary valvuloplasty. Indian Heart J 2001;53:773–775.
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- Zanchetta M, Colonna S, Rigatelli G, et al. Combined catheter-based pulmonary valvuloplasty and atrial septal defect closure in Noonan syndrome. A case report and literature review. Minerva Cardioangiol 2002;50:383–388.
- Fonseca N, Anjos R, Teixeira A, et al. Combined percutaneous treatment for pulmonary valve stenosis and atrial septal defect in an adult patient. Rev Port Cardiol 2003;22:107–114.
- Gupta M, Juneja R, Saxena A. Simultaneous device closure of muscular ventricular septal defect and pulmonary valve balloon dilatation. Catheter Cardiovasc Interv 2003;58:545–547.
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- Celebi A, Yalçin Y, Erdem A, et al. Successful transcatheter balloon dilatation of coarctation of aorta and coil occlusion of patent ductus arteriosus in a single catheterization procedure. Turk J Pediatr 2007;49:94–97.
- Song ZY, Shu MQ, Hu HY, et al. Clinical efficiency and safety analysis of transcatheter interventional therapy for compound congenital cardiovascular abnormalities. Clin Cardiol 2007;30:518–521.
- Atiq M, Khan SA, Tipu FA, Amin Z. Combined treatment for multiple cardiac defects with interventional techniques. Pediatr Cardiol 2008;29:890–893.
- Cheng SQ, Liu JP, Sun X, et al. Analysis and follow-up study on 8 children with combined congenital heart disease treated with simultaneous trans-catheter therapy. Zhongguo Dang Dai Er Ke Za Zhi 2008;10:599–602.
- Rao PS. Balloon aortic valvuloplasty. J Intervent Cardiol 1998;11:319–329.
- Xie C, Xia C, Zhao Z, Zhou Y. Hemodynamic features of congenital aortic valve stenosis with patent ductus arteriosus in children. Pediatr Int 2007;49:908–910.