Combined primary repair of right ventricular outflow tract obstruction and ascending aortic aneurysm Jinaga Nageswar Rao, Trushar Gajjar & Neelam Desai
Indian Journal of Thoracic and Cardiovascular Surgery ISSN 0970-9134 Indian J Thorac Cardiovasc Surg DOI 10.1007/s12055-014-0272-2
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Author's personal copy Indian J Thorac Cardiovasc Surg DOI 10.1007/s12055-014-0272-2
CASE REPORT
Combined primary repair of right ventricular outflow tract obstruction and ascending aortic aneurysm Jinaga Nageswar Rao & Trushar Gajjar & Neelam Desai
Received: 10 January 2012 / Revised: 23 June 2013 / Accepted: 5 August 2013 # Indian Association of Cardiovascular-Thoracic Surgeons 2014
Abstract A 23-year-old male presented with dyspnoea, cyanosis and clubbing and diagnosed to have the right ventricular outflow tract obstruction, patent foramen ovale, severe aortic regurgitation and ascending aortic aneurysm. The diagnosis was achieved by transthoracic echocardiography, cardiac catheterization and contrast-enhanced computed tomography. A successful intracardiac repair with aortic root replacement was performed. Postoperative course was uneventful. Postoperative and 3-month follow-up echocardiogram revealed no gradient across right ventricular outflow tract with mild pulmonary regurgitation and normally functioning prosthetic aortic valve. Histological examination of the aorta revealed cystic medial necrosis of aneurysmal sac wall. Keywords Right ventricular outflow tract obstruction . Ascending aortic aneurysm . Cystic medial necrosis
Introduction Ascending aortic aneurysm with severe aortic regurgitation (AR) having right ventricular outflow obstruction (RVOTO) and patent foramen ovale (PFO) leading to cyanosis and clubbing is very rare. A few cases of tetralogy of Fallot (TOF) and double-outlet right ventricle (DORV) with pulmonary stenosis having the dilatation of aorta and aortic regurgitation had been reported in the literature [1–3]. We report a J. N. Rao : T. Gajjar : N. Desai Sri Sathya Sai Institute of Higher Medical Sciences, District Anantapur, Prasanthigram 515134, Andhra Pradesh, India J. N. Rao (*) Department of Cardiothoracic and vascular surgery, Sri Sathya Sai Institute of Higher Medical Sciences, District Anantapur, Prasanthigram 515134, Andhra Pradesh, India e-mail:
[email protected]
case of a 23-year-old male with RVOTO, PFO with ascending aortic aneurysm and severe AR, who underwent successful infundibular resection, pulmonary valvotomy, RVOT reconstruction with transannular patch with closure of PFO and aortic root replacement.
Case report A 23-year-old male presented with dyspnoea on exertion and palpitation. Physical examination revealed, central cyanosis with clubbing, early diastolic murmur at aortic area and 3/6 ejection systolic murmur at the pulmonary area. Haematology showed haemoglobin 17.9 g% and packed cell volume 54.2 %. Preoperative room air saturation was 88 %. Electrocardiogram revealed, biventricular hypertrophy with strain pattern. X-ray chest showed cardiothoracic ratio of 60 %, dilated ascending aorta and reduced pulmonary blood flow. Transthoracic echocardiogram revealed severe AR, aortic annulus 31 mm, sinotubular junction 43 mm, aortic arch 33 mm, aorta at diaphragm 14 mm, severe infundibular and valvar pulmonary stenosis with a gradient of 134 mmHg, dysplastic pulmonary valve and left ventricular ejection fraction (LVEF) of 65 % (Fig. 1a, b). Contrast-enhanced computed tomography showed (Fig. 2a) ascending aortic aneurysm with aortic root 47 mm, ascending aorta 50 mm and arch of aorta 34 mm with no dissection or thrombosis. Cardiac catheterization showed no step up at right atrial or right ventricular level; Femoral artery saturation was 88.6 %, right ventricular pressure 124/8 mmHg, pulmonary artery pressure 14/6 mmHg and left ventricle pressure 168/10 mmHg. Right ventriculogram showed (Fig. 2b) severe valvar and infundibular pulmonary stenosis with post stenotic dilatation of main pulmonary artery and good-sized confluent pulmonary arteries. Aortic root injection showed grade III/IV AR, dilated aortic root and ascending aorta with normal coronaries. The
Author's personal copy Indian J Thorac Cardiovasc Surg
Fig. 1 a Transthoracic echocardiogram in parasternal short axis view showing dilated aortic root with non-coapting leaflets, thickened and dysplastic pulmonary valve. b Colour Doppler in the RVOT shows turbulence with gradient across the RVOT
surgical approach was through a conventional median sternotomy, and the cardiopulmonary bypass was established with an aortic and bicaval cannulation. Patient was cooled to 24 °C. The aorta was opened vertically which was thin walled and dilated without any dissection flap. Aortic valve was bicuspid and rolled up non-coapting leaflets with dilated annulus. The aortic valve with aneurysm was excised and the root was replaced with 25 St. Jude aortic valved graft conduit using modified Bentall technique. The pulmonary artery was opened vertically, and the pulmonary valvotomy was done. The right ventricular outflow tract (RVOT) was like a narrow tunnel. It was cut across the pulmonary annulus with preservation of two pulmonary cusps and augmented with autologous untreated pericardial patch. The PFO was closed directly. Postoperative course was uneventful and echocardiogram revealed a normally functioning prosthetic aortic valve, gradient across aortic valve was 10/6 mmHg, no RVOT gradient, mild pulmonary regurgitation, right ventricular systolic pressure was 18 mmHg, good right ventricular function and LVEF 30–35 %. The histopathological examination showed cystic medial necrosis of aneurysm wall. At 3-month follow-up, the
patient was asymptomatic and echocardiogram showed no RVOT gradient, mild pulmonary regurgitation and good biventricular function and the gradient across aortic valve 22/15 mmHg.
Discussion From the review of literatures, cases like TOF and DORV with dilated ascending aorta having cyanosis [1–3] and cases of giant aneurysms of ascending aorta compressing RA and pulmonary artery producing cyanosis [2, 3] have been described. But ours is the first case of ascending aortic aneurysm with isolated pulmonary stenosis in the absence of a ventricular septal defect (VSD) presented with cyanosis. The aortic root and the ascending aortic dilatation occurs in TOF due to haemodynamic stress as a result of increased blood flow through the aorta and also due to previous aortopulmonary shunts, or it may occur secondary to congenital abnormalities of the aortic valve and acquired infective endocarditis [1–3]. The pre-existing histologic abnormalities in the media of the
Fig. 2 a CECT scan showing dilated aortic root with ascending aortic aneurysm. b Right ventriculogram showing infundibular and valvar pulmonary stenosis
Author's personal copy Indian J Thorac Cardiovasc Surg
aortic root and the ascending aorta may also contribute to the aortic root dilatation [4]. The important underlying mechanisms for aneurysm formation might be the apoptosis, particularly in patients with bicuspid aortic valves or the cellular abnormalities in patients with conotruncal anomalies [3]. There are many causes of cyanosis with ascending aortic aneurysm like large ascending aortic aneurysm causing right atrial or pulmonary artery compression giving rise to cyanosis [2, 3], and the dilatation of aorta with aortic regurgitation have been described in patients of tetralogy of Fallot [5]. The cause of cyanosis in our case was RVOTO with PFO, different from other causes described in the literature. The diagnosis was primarily made by transthoracic echocardiography. The combined primary replacement of the aortic root and ascending aorta along with the relief of RVOTO and closure of the PFO has successfully relieved the symptoms. The RVOT was tunnel like and without obvious muscle bundles in our case which required RVOT augmentation using transannular patch. Histopathological findings in our patient showed medial degeneration of the aortic wall. The effects of chronic hypoxia and other factors in the process of medial degeneration associated with aortic dilation and aortic regurgitation in TOF and like situations are yet to be defined [5]. At 3-month follow-up, patient was asymptomatic and echocardiography revealed good outcome.
Conclusion The ascending aortic aneurysm with severe AR having RVOTO and PFO leading to cyanosis and clubbing is a very rare combination. Transthoracic echocardiography is an important non-invasive diagnostic modality. In spite of being complex and technically challenging anatomy, the immediate postoperative results and early outcome were satisfactory.
References 1. Capelli H, Ross D, Somerville J. Aortic regurgitation in tetrad of Fallot and pulmonary atresia. Am J Cardiol. 1982;49:1979–83. 2. Nagao GI, Daud GI, McAdams AJ, Schwartz DC, Kaplan S. Cardiovascular anomalies associated with tetralogy of Fallot. Am J Cardiol. 1967;20:206–15. 3. Dodds III GA, Warnes CA, Danielson GK. Aortic valve replacement after repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot. J Thorac Cardiovasc Surg. 1997;113:736–41. 4. Tan JL, Davlouros PA, McCarthy KP, Gatzoulis MA, Ho SY. Intrinsic histological abnormalities of the aortic root and ascending aorta in tetralogy of Fallot: evidence of causative mechanism for aortic dilatation and aortopathy. Circulation. 2005;112:961–8. 5. Downing WD, Kouchoukos NK. Ascending aortic aneurysm. In: Edmunds LH, editor. Cardiac surgery in the adult. 1st ed. New York: McGraw-Hill; 1997. p. 1165–95.
