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Tex Heart Inst J. 2003; 30(2): 152–154.
PMCID: PMC161906

Unruptured Aneurysm of the Sinus of Valsalva

Presenting with Right Ventricular Outflow Obstruction
Roy Thankachen, MCh, Roy Gnanamuthu, MCh, Harikrishna Doshi, MCh, Vinayak Shukla, MCh, DNB, and Roy John Korula, MCh, FIACS, FACS

Abstract

Sinus of Valsalva aneurysms rarely present until rupture occurs. We describe the case of a patient who presented with acute right heart failure and upon investigation was found to have an unruptured sinus of Valsalva aneurysm causing right ventricular outflow obstruction; there was an associated subaortic ventricular septal defect. To the best of our knowledge, only 1 other case with these features has been reported in the medical literature.

The diagnosis was made by transthoracic echocardiography and cardiac catheterization. Through an aortic and right ventricular approach, we successfully excised the aneurysmal right coronary sinus, closed the ventricular septal defect, and replaced the aortic valve. (Tex Heart Inst J 2003;30:152–4)

Key words: Aortic aneurysm/complications/diagnosis/pathology, aortic valve insufficiency/complications/diagnosis/pathology, heart septal defects, ventricular, heart valve diseases/diagnosis, ventricular outflow obstruction/etiology, sinus of Valsalva/surgery, ventricular septal defect

Aneurysms of the sinus of Valsalva rarely present with symptoms unless rupture occurs. However, there have been a few reported cases of unruptured sinus of Valsalva aneurysms, which have presented with conduction disturbances, myocardial ischemia, and symptomatic cardiac dysfunction. 1 We describe a patient with an unruptured aneurysm of the sinus of Valsalva associated with a ventricular septal defect, who presented with right ventricular outflow obstruction and acute right ventricular failure.

Case Report

In August 2001, a 32-year-old man presented to us with a 2-year history of chest pain and mild dyspnea on exertion. During the past 10 days, he had experienced an acute exacerbation of these symptoms, together with progressive jaundice. On clinical examination, he was found to be markedly icteric, with hepatomegaly and ascites. Auscultation revealed severe aortic regurgitation and severe tricuspid regurgitation.

On transthoracic echocardiography, he was found to have a large aneurysm of the right coronary sinus of Valsalva with obstruction of the right ventricular outflow tract, severe aortic and tricuspid regurgitation, and a small subaortic ventricular septal defect. Cardiac catheterization showed a large aneurysm that originated from the right coronary cusp and extended into the right ventricular outflow tract, almost completely occluding it (Fig. 1). The peak systolic right ventricular pressure was 86 mmHg and the peak pulmonary artery pressure was 13 mmHg; there was a right ventricle-to-pulmonary artery gradient of 54 mmHg. Oxygen saturation measurements showed no significant increase. An abdominal ultrasonogram revealed marked hepatic venous congestion and ascites.

figure 17FF1
Fig. 1 Left ventricular angiogram shows the aneurysm of the right coronary cusp extending into the right ventricular outflow tract.

At surgery, the above findings were confirmed. The right coronary sinus, which was aneurysmal and full of clots, was obstructing the right ventricular outflow tract from inside and had caused thinning of the right ventricular free wall. This aneurysm almost completely obstructed the right ventricular outflow tract (Fig. 2). The right cusp of the aortic valve, thickened and fibrosed, partly occluded the ventricular septal defect, which lay below the right coronary sinus.

figure 17FF2
Fig. 2 Drawing of the aneurysm (A) arising from the right coronary cusp (RRC).

Through an aortic and right ventricular approach, we excised the aneurysmal right coronary sinus, together with part of the thinned right ventricular wall. We repaired the ventricular septal defect with a Dacron patch, which we extended to the aorta to repair the sinus of Valsalva. The right ventricular outflow tract was repaired with a small pericardial patch. We replaced the aortic valve with a 19-mm St. Jude Medical bileaflet prosthetic valve (St. Jude Medical, Inc.; St. Paul, Minn).

Postoperatively, the patient had an uneventful recovery, and he was well on his most recent follow-up, 20 months later (March 2003).

Discussion

Sinus of Valsalva aneurysms usually remain asymptomatic and undetected, unless rupture occurs. In rare instances, unruptured aneurysms of the right sinus of Valsalva protrude into the right ventricular outflow tract and cause obstruction. When this happens, patients may present with symptoms of acute right heart failure, as did our patient. There have also been a few reported cases in which patients with an unruptured aneurysm of the sinus of Valsalva have presented with exertional dyspnea, palpitations, and angina-like chest pain. 2

In most cases, the aneurysm arises from the right coronary cusp, which is normally adjacent to the right ventricular outflow tract. These aneurysms may be congenital, due to a deficiency in the muscular and elastic tissues at the base of the aorta, or acquired after infective endocarditis, atherosclerosis, or aortic dissection. 3 Unruptured aneurysms of the right coronary cusp have been found to cause pulmonary insufficiency (by distorting the pulmonary valve leaflets), myocardial ischemia (by obstructing the right coronary ostia), conduction disturbances, and tricuspid incompetence. 1 A coexisting ventricular septal defect has also been reported. 4 In such an event, the ventricular septal defect is situated immediately below the aneurysm. This association has been explained by the structural abnormality in the distal bulbar septum. 5

Two-dimensional and Doppler echocardiography can adequately show unruptured sinus of Valsalva aneurysms. 6 However, an associated ventricular septal defect may occasionally escape detection if the wall of the aneurysm or the prolapsing cusp of the aortic valve occludes it.

The surgical approach to repair depends on various factors, such as whether the aneurysm is ruptured, the need to repair or replace the aortic valve, and the presence or absence of a ventricular septal defect. In our patient, a bicameral approach through the aorta and the right ventricular outflow tract was used to enable valve replacement, ventricular septal defect closure, and outflow tract repair.

Conclusions

Cases of unruptured sinus of Valsalva aneurysm with right ventricular outflow obstruction in association with subaortic ventricular septal defect are extreme-ly rare. To our knowledge, only 1 other such case has been reported. 4 Our patient presented with symptoms of acute right ventricular failure. A good prognosis depends on early diagnosis and prompt surgical intervention. A bicameral operative approach is probably the best option in such patients.

Footnotes

Address for reprints: Dr. Roy John Korula, MS, MCh (Thor), FIACS, FACS, Professor & Head, Department of Cardiothoracic Surgery, Christian Medical College & Hospital, Vellore – 632 004, India

E-mail: moc.liamtoh@alurokyor

References

1. Bulkley BH, Hutchins GM, Ross RS. Aortic sinus of Valsalva aneurysms simulating primary right-sided valvular heart disease. Circulation 1975;52:696–9. [PubMed]
2. Liau CS, Chu IT, Ho FM. Unruptured congenital aneurysm of the sinus of Valsalva presenting with pulmonary stenosis. Catheter Cardiovasc Interv 1999;46:210–3. [PubMed]
3. Marques KM, De Cock CC, Visser CA. Isolated unruptured aneurysm of the right sinus of Valsalva causing right ventricular outflow obstruction. Heart 1999;81:447–8. [PMC free article] [PubMed]
4. Liang CD, Chang JP, Kao CL. Unruptured sinus of Valsalva aneurysm with right ventricular outflow tract obstruction associated with ventricular septal defect. Cathet Cardiovasc Diagn 1996;37:158–61. [PubMed]
5. Kirklin JW, Barratt-Boyes BG. Congenital aneurysm of the sinus of Valsalva. In: Cardiac surgery. Morphology, diagnostic criteria, natural history, techniques, results, and indications. 2nd ed. New York: Churchill Livingstone; 1993. p. 826.
6. Desai AG, Sharma S, Kumar A, Hansoti RC, Kalke BR. Echocardiographic diagnosis of unruptured aneurysm of right sinus of Valsalva: an unusual cause of right ventricular outflow obstruction. Am Heart J 1985;109:363–4. [PubMed]

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