Successful Percutaneous Balloon Valvuloplasty for Combined Congenital Tricuspid and Pulmonary Stenosis

 

 

 

 

 

Nawal Azhari, MBChB, Al Fadley Fadel, M.D., Galal Omar, M.D.

 

Percutaneous balloon valvuloplasty was successfully performed in a 13-month-old symptomatic infant with congenital tricuspid and

pulmonic valve stenosis.  Both valves were dilated in the same setting, resulting in a significant hemodynamic and clinical improvement.

 

J Interven Cardiol 1998; 11:73-76

 

Introduction

 Since introduced by Kan and her collegues in 1982,1  percutaneous balloon valvuloplasty is considered the treatment of choice for congenital pulmonic stenosis.  There have been only seven previous case reports of balloon valvuloplasty in tricuspid valve stenosis, all in adult patients with rheumatic fever.2-8  Three case reports of  PBVP of carcinoid tricuspid valve have been reported, 9-11 also in adult patients, and two were in combination with carcinoid pulmonary stenosis. 10-11 To our knowledge, there have been only two case reports of PBVP in adults for congenital tricuspid stenosis 12,13; one was in combination with congenital pulmonary stenosis following open valvotomies for both tricuspid and pulmonic stenosis, 13 and the other 12 was for isolated tricuspid stenosis. We report the first case of concurrent PBVP for congenital tricuspid and pulmonic stenosis.

 

Case Report

 A 13-month-old, 8-kg, male infant was referred to our center for evaluation of cyanosis and easy fatigability of 6 months duration. Examination revealed an oxygen saturation in room air of 84% at a heart rate of 109/min. The neck veins were not distended and there was no peripheral edema.  The peripheral pulses were normal.  The liver was enlarged 3 cm below the right costal margin.  Cardiac auscultation revealed a harsh grade IV-VI systolic ejection murmur loudest at the base of the heart and a grade II/VI diastolic rumble along the left lower sternal border. The electrocardiogram (ECG) showed sinus rhythm, right atrial enlargement, and right axis deviation with right ventricular hypertrophy.  The X-ray of the chest showed a small heart with oligemic lungs, levocardia and situs solitus. The two dimensional echocardiogram revealed a dilated right atrium and right ventricle with a stretch patent foramen ovale with a right to left shunt. The tricuspid valve leaflets were thickened with doming in diastole. The tricuspid valve annulus measured 1.75cm. Doppler evaluation revealed tricuspid valve stenosis with a peak gradient of 18mmHg and mean gradient of 10mmHg. There was moderate tricuspid valve incompetence with a tricuspid regurgitation jet velocity of 4m/sec. There were also thickened and doming pulmonary valve leaflets with a peak gradient of 52mmHg and mean gradient of 35 mmHg. The pulmonary valve annulus measured 1.2 cm. Left ventricular systolic function was normal with an ejection fraction of 63% and shortening fraction of 32%.

The diagnosis of congenital combined tricuspid and pulmonary valve stenosis with moderate tricuspid regurgitation was made, and the patient was taken for cardiac catheterization with the intention of ballooning both the pulmonary and tricuspid valves.

 

Cardiac Catheterization and Procedure

The procedure was performed under general anesthesia and 100% oxygen. Hemodynamic data were obtained (Table 1).Thetricuspid valve gradients were measured by simultaneous recording of right ventricular and right atrial pressures pre- and post

balloon dilatation. Cardiac output was measured by the Fick principle, and the valve area was calculated using the Gorlin Formula.         Because of the tricuspid valve stenosis, the significant tricuspid valve regurgitation, and the presence of a markedly dilated atrium, we elected to access the tricuspid valve through the neck to facilitate free manipulation and positioning of the balloon catheter across the tricuspid valve.

The co-existence of pulmonary valve stenosis and tricuspid valve stenosis with regurgitation raised the concern of possible worsening of the tricuspid valve regurgitation following dilatation of the tricuspid valve stenosis. For that reason, the pulmonary valve dilatation was performed first ( Fig. 1A, and only after confirming a successful pulmonary valvuloplasty ( gradient drop from 55 to 25 mmHg) was the tricuspid valve dilatation performed. A 5Fr, 12-mm, 3-cm balloon catheter was used to dilate the pulmonary valve. Following that, the tricuspid valve was dilated using a 7Fr, 15-mm, 3-cm balloon catheter ( Fig.1B). The pressure gradient across the pulmonary valve dropped from 52 to 25 mmHg and the pressure gradient across the tricuspid valve dropped from 18 to 10 mmHg. Despite the obvious decrease in pressure gradients, there was nearly no change in the mean right atrial pressure, which is most likely due to the presence of significant tricuspid regurgitation that was present prior to balloon dilatation, as well as to the high right ventricular end-diastolic pressure ( which is probably secondary to pulmonary regurgitation). Aortic saturation rose from 84% to 94 %, which suggest a decrease in the right to left shunt at the atrial level.

Cardiac output increased from 0.8 to 1.0 L/min and the tricuspid valve area increased from 0.2 to 0.4 cm2. Right ventricular cineangiography at the end of the procedure showed no increase in the degree of tricuspid regurgitation. No complication was encountered, and the patient was discharged the following day. At 6-month follow-up the child was asymptomatic. Repeat echocardiogram showed that the peak gradient across the tricuspid valve was 10 mmHg and the mean gradient was 5mmHg (as compared to a gradient of 18 mmHg and 10 mmHg, respectively, prior to PBVP). The tricuspid valve leaflets opened well with a  normal Doppler flow pattern. There was moderate tricuspid valve regurgitation with a jet velocity of 3.2 m/sec.  The right atrium remained dilated. The pressure gradient across the pulmonic valve was 25mm Hg. The right ventricular pressure was 40mmHg.

 

 

Table 1.  hemodynamics Before and After Balloon Valvuloplasty

 

 

Parameter

 

 

Basal

 

Post Pulmonary Valvuloplasty

Final Results

(Post tricuspid Valvuloplasty)

RA Pressure

27/23 (20)

            (18)

24/17  (18)

RV Pressure

70/7

48/8

48/8

PA Pressure

20/10 (21)

24/10   (22)

24/20  (22)

AO Pressure

87/54 (68)

105/70 (84)

100/65 (80)

RA = right atrium; RV= right ventricle; LV= left ventricle; AO= aorta

Pressures are given in mmHg; ( ) = mean pressure

 

Discussion

Congenital valve stenosis is relatively common, but isolated congenital tricuspid valve stenosis is quite rare. The combination of isolated pulmonic and tricuspid stenosis in the presence of a normal sized right ventricle is even more unusual.

 

PBVP has become the treatment of choice for isolated pulmonary valve stenosis since 1982.1 This report confirms the feasibility of PBVP for the treatment of combined pulmonary and tricuspid stenosis in a young child. This combined approach was first reported by Chen et al. in 1988 in a 55-year-old patient.13  In our case report, apart from the unusual combination of valvular stenosis, the age of presentation makes this case very unique. The child presented with cyanosis, which is attributable to the severe tricuspid valve stenosis and right to left shunt across the stretched foramen ovale. Echocardiography confirmed the diagnosis of moderate tricuspid valve regurgitation and moderate to severe tricuspid valve stenosis with thick and rolled-in tips of the tricuspid valve leaflets, suggestive of a dysplastic tricuspid valve.  Because of the co-existence of pulmonary valve stenosis and tricuspid valve stenosis with regurgitation, addressing the tricuspid valve stenosis first may worsen the tricuspid valve regurgitation, particularly if pulmonary valvuloplasty fails to reduce the right ventricular pressure.  For that reason, we decided to dilate the pulmonary valve first, and only if this were successful would the tricuspid valvuloplasty be attempted.

 

Because of the co-existing stenosis and regurgitation of the tricuspid valve, we thought it would be much easier to obtain access through the neck rather than through the femoral vein.  Overall, this child has benefited from the balloon dilatation, as shown by the hemodynamic data and by the clinical improvement and relief of cyanosis.  However, the tricuspid valve still looks abnormal and is still significantly regurgitant.    Because of the overall improvement, the plan is to continue observing this child conservatively.

 

 In conclusion, our report shows that in the case of a congenital combined tricuspid and pulmonic valvular stenosis it is technically possible to dilate both valves and thus postpone any possible surgical intervention for a later date.

 

 


Acknowledgment:  We would like to thank Marie Ah-Leong  for her secretarial assistance in preparing this manuscript.

 

 


References:

 

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13.     Chuan RC, Zheng XI, Zheng DH, et al. Concurrent PBV for combined tricuspid and pulmonary stenosis.  Cathet Cardiovasc Diagn 1988; 15:  55-60.