Asian Cardiovasc Thorac Ann 2006;14:93-97
© 2006 Asia Publishing EXchange Ltd
Aortic Regurgitation with Ventricular Septal Defect in the Young
Kaushalendra Singh Rathore, MCh
Department of Cardiothoracic and Vascular Surgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
For reprint information contact: Kaushalendra Singh Rathore, MCh Tel: 91 98 9295 5794 Fax: 91 22 2407 6100 Email: kaushalendra_rathore{at}hotmail.com, Dept CVTS, KEM Hospital and Seth GS Medical College, Mumbai 400012, India.
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ABSTRACT
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This study was carried out to assess the outcome in patients who had aortic valve replacement compared to those who underwent aortic valve repair for aortic regurgitation associated with a ventricular septal defect. Of 300 patients undergoing ventricular septal defect closure between May 1990 and December 2003, 36 (12%) had moderate to severe aortic regurgitation; 7 underwent concomitant aortic valve repair and 29 had aortic valve replacement. The mean age of these 36 patients was 17.72 ± 6.84 years, and 69% were male. Follow-up was 8.20 ± 4.97 years in the valve replacement group and 4.1 ± 0.8 years in the valve repair group. The freedom from re-operation after valve repair was 76% after 4 years. After one year of follow-up in 35 patients, 27 were in New York Heart Association class I (77%) and 8 were in class II (23%). After 8 years, 12/21 (57%) patients were in class I, 5 (24%) in class II, and 2 (10%) in class III. Of 22 patients who had a dilated left ventricle, 15 regained normal left ventricular function and volume. Valve repair is preferred, but increasing age makes valve replacement a better alternative.
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INTRODUCTION
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The association of ventricular septal defect (VSD) with aortic regurgitation (AR) is well documented. In our part of the world where subpulmonic or doubly committed VSD are frequent, the incidence of associated aortic valve prolapse and regurgitation is high. There is controversy as to whether the optimal treatment is aortic valve replacement or valve repair, and also regarding the timing of surgery as patients are often referred late. We conducted this retrospective study to analyze the factors leading to the decision to repair or replace the aortic valve in patients who were operated on at our Institute.
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PATIENTS AND METHODS
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Between May 1990 and December 2003, 300 patients underwent surgery for VSD closure. Of these, 36 (12%) had associated AR. The AR was regarded as mild if the regurgitation jet reached 25% of the distance from the left ventricular (LV) apex, moderate if it reached 50% of this distance, and severe if more than 50% of the distance. The patients were serially followed up by transthoracic 2-dimensional echocardiography with a Hewlett-Packard Sonos 5500 (Hewlett Packard, Inc., Anaheim, CA, USA). Ventricular septal defect was classified according to the Congenital Heart Surgery Nomenclature and Database Project.1 Aortic valve function was defined as normal when there was no AR or valve prolapse. The severity of regurgitation and prolapse was graded by angiography and echocardiography.2,3 Aortic regurgitation was regarded as mild if the jet did not reach the tip of the anterior mitral leaflet, moderate if it reached the tip, and severe if it went beyond the tip. Aortic valve prolapse was mild if there was buckling of the aortic cusp with minimal herniation during systole, moderate if there was obvious herniation into the VSD, and severe if the sinus herniated into the right ventricular outflow tract during systole and diastole. New York Heart Association (NYHA) criteria were used to determine functional status.
Surgery was performed under cardiopulmonary bypass with standard aortic-bicaval cannulation and moderate hypothermia. Cold antegrade blood ostial cardioplegia was used. After aortic crossclamping, an aortotomy was made, and the aortic valve was inspected. The VSD was approached through the right ventricle, aortotomy, tricuspid valve, or pulmonary artery, depending on its location. All VSDs were closed with a Dacron patch, except one that was closed directly. Interrupted polypropylene sutures were used in all patients operated on before 1996. From 1997, continuous polypropylene sutures were used for VSD closure. Valve repair was carried out by Trusler’s technique.4 When severe AR and prolapse was found with a lack of coaptation and fibrosis of the valve leaflets, aortic valve replacement was undertaken with interrupted 2/0 Ethibond (Ethicon, Inc., Somerville, NJ, USA) sutures with Teflon pledgets placed above the ring. A valve size of 21 mm or more was used in all patients except for 3, in whom a 19 mm valve was used. Oral anticoagulant (acenocoumarol) and aspirin were started from the first postoperative day, and the international normalized ratio was maintained between 2 and 3. Echocardiography was performed during the follow-up of patients known to have diastolic dysfunction. Right ventricular diastolic dysfunction was defined as antegrade late diastolic flow in the pulmonary artery, where atrial contraction was transmitted directly to the pulmonary artery, and the stiff right ventricle acted as a conduit.5
The data in the records of these patients were analyzed with Epi-Info Version 6.01 statistical software (Centers for Diseases Control and Prevention, Atlanta, GA, USA).
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RESULTS
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The ages of the 36 patients with AR ranged from 5 to 32 years (mean, 17.72 ± 6.84 years) and 69% were male (Table 1
). All patients were in normal sinus rhythm. The follow-up period ranged from 6 months to 12 years (mean, 8.20 ± 4.97 years). Preoperatively, 17 patients (47%) were in NYHA class II, 16 (44%) were in class III, 2 were in class I, and 1 was in class IV. Catheterization was undertaken in 16 patients, 4 had a pulmonary-to-systemic blood flow ratio (Qp/Qs) > 2:1. Echocardiography data are given in Table 2. The majority of patients had a subpulmonic (doubly committed) type of VSD. All doubly committed VSDs were restrictive. Of the 4 patients with a nonrestrictive VSD, 2 had severe prolapse and 2 had mild prolapse. The VSD was closed through the right ventricle in 15 patients, via a transaortic approach in 5, a transpulmonary approach in 6, transaortic and right atrial approach in 5, and through the right atrium in 5. One patient had two VSDs: one subaortic and an additional muscular VSD. The diameters of the VSDs varied from 5 mm to 2.5 cm. Preoperative echocardiography was useful to determine the anatomical location of the VSD.
Of 17 patients with aortic valve prolapse, 13 (76%) had a right cusp prolapse that formed the superior margin of the VSD. The prolapse was severe in the restrictive juxta-aortic type of VSD, and the severity increased with age. No correlation was found between the type of VSD and the leaflet that had prolapsed. The aortic valve was tricuspid in all patients. One patient developed mild AR in the immediate postoperative period, and 2 (aged 12 and 14 years at surgery) developed mild AR after 5 years of follow-up. Cardiopulmonary bypass time ranged from 70 to 120 min (mean, 98 ± 13 min), aortic crossclamp time ranged from 50 to 98 min (mean, 68 ± 11 min).
There was no perioperative mortality. One patient developed complete heart block requiring pacemaker insertion, and one had first-degree heart block that converted to sinus rhythm after temporary pacing for a week. Four patients developed pericardial effusion; 2 required subxiphoid tube drainage. All patients were discharged in NYHA class I. No patient had right ventricular diastolic dysfunction in the immediate postoperative period.
There were no valve-related complications during follow-up. After one year of follow-up in 35 patients, 27 (77%) were in NYHA class I and 8 (23%) were in class II. Two deaths occurred in late follow-up: one after 3 years because of leiomyosarcoma of the stomach, and one after 6 years because of cardiac failure. After 5 years of follow-up in 31 patients, 18 (58%) were in NYHA class I, 12 (39%) were in class II, and one (3%) was in class III. After 8 years, 12/21 patients (57%) were in NYHA class I, 5 (24%) in class II, and 2 (10%) in class III; 2 were lost to follow-up. Six patients completed their 10-year follow-up: 3 were in class I, 1 was in class II, and 2 were in class III (Figure 1); the 2 patients in class III had ejection fractions < 30% preoperatively. Of the 22 patients who had a dilated left ventricle, 15 regained normal LV function and volume (43.56 ± 2.34 mL). The other 7 patients were in functional class II/III. Freedom from re-operation in the valve repair group was 76% after 4 years.
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DISCUSSION
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The incidence of AR with VSD is 4% – 5% in the Western population, but it is higher in Orientals.3,4 In our study, the incidence was 12%, which can be explained by the fact that many patients were from the north-eastern part of the country, with a mixed Oriental and Caucasian population. Two etiological factors are the lack of aortic valve support and hemodynamic changes.6 Aortic regurgitation is more common with subarterial VSD, especially with subpulmonary VSD.7,8 Our finding of 32 subpulmonic or doubly committed VSDs and 4 perimembranous VSDs strongly supports this. We were unable to find any correlation between the type of VSD and the cusp that prolapsed; in all types of VSD, right cusp prolapse was predominant. The Venturi effect may be an important phenomenon in the progression of regurgitation and prolapse of the aortic valve leaflets because of a restrictive VSD.9 Restrictive VSD leads to slow progression of AR which peaks at 5 to 8 years.9 As the prolapse increases, the leaflets fall into the VSD, further reducing the effective orifice area.
The prolapsing aortic valve is initially mobile but with time (3–4 years) it becomes immobile.7 One study concluded that there was no need for valve repair in mild to moderate prolapse.8 We consider AR and valve prolapse to be progressive, and it is clear that the severity of regurgitation and prolapse was greater in adult patients. Some Doppler echo studies suggest an age-related increase in the incidence of AR signals in structurally normal adult hearts.3 Univariate analysis showed age to be a significant risk factor for repair failure. In our study, 12 patients had right coronary cusp prolapse, described by Trusler and colleagues4 as a right commissural defect. A VSD between the right coronary cusp and the noncoronary cusp is most often associated with a structural defect.
The reason for the late age of presentation in our study was probably late referral. Of 7 patients in whom valve repair was performed, 3 (43%) developed mild to moderate AR on follow-up of 5 years. Aortic valve prolapse leads to failure of the procedure at 10 years, and older age is a significant risk factor.9 Patients with pulmonary arterial hypertension rarely develop cusp prolapse or regurgitation.10 Only one of our patients had severe pulmonary arterial hypertension, and 2 had a moderate degree. Ventricular septal defect repair was performed via a right ventriculotomy in 15 patients, mainly due to the surgeon’s preference; all were operated on before 1997. Since then, all VSDs were closed through the right atrium or the aorta. No diastolic dysfunction or arrhythmias were detected in the follow-up. Plication as described by Trusler and colleagues4 was used in our patients. Another approach advocated by Carpentier11 is wedge excision of the central part of the leaflet with subsequent re-approximation by suturing. Yacoub and colleagues7 reported valve repair through a transaortic approach. Other methods of repair have been devised with reasonable results.10,12,13 Due to poor results with mechanical valve replacement in children, the Ross procedure has been used to treat VSD with associated AR; however, if the VSD is subaortic, the Ross procedure is not suitable.14 Okita and colleagues15 found the risk factors to be older age, greater cardiothoracic ratio, perimembranous VSD, and multiple previous valvuloplasties.
We consider aortic valve replacement a preferred option in patients with moderate to severe AR and a high cardiothoracic ratio, dilated left ventricle, LV dysfunction, and older age. In young patients, valve repair is preferred as biological valves have a high rate of degeneration, perhaps because of accelerated calcium metabolism, leading to re-operation.16 This subset of patients can benefit from mechanical valve replacement rather than homograft or valve repair. Our patients had good postoperative functional status after 5 years of follow-up; the functional capacity deteriorated thereafter. According to Basarir and colleagues,17 the improvement in LV structure and function postoperatively depends on the stage at surgery. The LV end-diastolic and end-systolic volumes fall with increased ejection fraction within 6 months postoperatively. However, patients with preoperative LV dysfunction showed poor results postoperatively. The most likely explanation is their greater age at the time of surgery. The valvular condition that deteriorates with age ultimately leads to decompensation of LV function. A 21 mm mechanical heart valve provides good hemodynamic performance, but the 19 mm size can also be sufficient for patients with a small body surface area.18 In 3 of our patients with a small BSA, a 19 mm valve was inserted to avoid performing high-risk aortic root enlargement procedures at an early age.
The mean age in our study was 17 years, and by this age, the leaflets have become thick and fibrotic, making valve repair difficult. Rao and colleagues18 reported failure of valve repair leading to valve replacement in 56% of patients at 5 years. This chronic disease leads to structural changes in the valve leaflets that make repair prone to failure. The functional outcome can be predicted after one year of follow-up. Patients who remained in NYHA class I continued to be functionally stable, while those in a higher NYHA class after one year deteriorated during the follow-up. Whatever changes occur in the left ventricle, start in the first postoperative year. Added to all these problems, valve repair may increase the morbidity and mortality in these patients. Based on clinical and echocardiographic data, we found that valve repair produced the best results before 7 years of age. After this age, valve pliability decreases, severity of prolapse and regurgitation increases, and with advancing age, valve repair is more vulnerable to failure. A subpulmonic VSD requires prompt repair even if the Qp/Qs is less than 2. Both modes of treatment are appropriate when applied at the right time in the clinical spectrum. Increasing age makes results of valve repair suboptimal, and in these cases, valve replacement is a better option.
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REFERENCES
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ABSTRACT
INTRODUCTION
