Use of Dobutamine Stress Echocardiography for Periprocedural Evaluation of a Case of Critical Valvular Pulmonary Stenosis with Delayed Presentation.

We report a case illustrating a 39-year-old man with delayed presentation of severe pulmonary valve (PV) stenosis, clinical evidence of congestive right heart failure in the form of enlarged liver, raised jugular venous pressure, and anasarca without cyanosis. Echocardiography (echo) was used both for diagnosis and monitoring this patient as main tool. The contractile reserve of the right ventricle (RV) was evaluated by infusion of dobutamine and diuretic for 4 days before pulmonary balloon valvotomy. Both the tricuspid annular peak systolic excursion and diastolic (diastolic anterograde flow through PV) function of RV improved after percutaneous balloon pulmonary valvotomy. These improvements were clinically apparent by complete resolution of anasarca, pericardial effusion, and normalization albumin-globulin ratio. The periprocedural echo findings were quite unique in this illustration.

INTRODUCTION

Adults with congenital heart diseases usually have three types of presentation such as accidental detection, some symptoms, or with very critical illness. Critical pulmonary valve stenosis (PS) may present very late as atrial septal defect and aortic coarctation because of unique adaptability of the right ventricle (RV) to the extremes of pressure and volume overload. Valvular PS with right ventricular dysfunction without cyanosis is rare presentation in an adult. Earlier intervention or surgery improves right ventricular function by reduced right ventricular outflow tract (RVOT) obstruction, restored contractile reserve, and extensive favorable remodeling of RV similar to aortic valve replacement done for severe aortic stenosis with left ventricular dysfunction (low flow-low gradient phenomenon with good contractile reserve). Rare periprocedural complications such as suicidal RV, flash pulmonary edema, rupture of the RVOT, and further worsening right heart failure need special attention in these patients.[123] At present, percutaneous balloon pulmonary valvotomy (PBPV) is the standard of care in such condition.

CASE REPORT

A 39-year-old male presented with breathless on exertion and anasarca for the last 6 months. He had undergone tapping of ascites twice without any relief of symptoms. He was an apparently healthy manual labor, married and having four children. There was no family history of congenital heart disease and consanguineous marriage. On inspection, anasarca and significantly raised jugular pressure were characteristic. Raised jugular venous pressure marked by fullness of angle mandible even on standing. There was no cyanosis or clubbing. Room air SPO2 was 96%. Room right radial artery and right antecubital vein O2 saturation were 97% and 70%, respectively. Right radial PO2 was 85 mmHg. On clinical examination, heart rate was 114/min regular, respiration rate was 17/min, and right arm sitting blood pressure was 110/60 mmHg. Apex was diffuse and pulmonary ejection click was not audible. There was Grade III/VI ejection systolic murmur with selective radiation to left shoulder and soft P2. Abdominal examination revealed tense ascites and scrotal edema. Chest X-ray in (anterior-posterior) AP view showed situs solitus levocardia with D-loop ventricles, cardiothoracic ratio of 0.8, right ventricular apex, enlarged right atrium (RA), elevated diaphragm, and pericardial effusion. Twelve-lead electrocardiogram (ECG) showed right ventricular hypertrophy (RVH), right atrial enlargement, Qr pattern in V1–V2, tall R-waves in V1–V6 leads with strain pattern, and no S-wave in V4–V6 [Figure 1a]. Therefore, significant RVH and right ventricular dilatation were suspected. A repeat 12-lead ECG was repeated keeping V4–V6 leads in V7–V9 position. This modification in the position of ECG leads disclosed R-wave voltage in the most posterior leads of <12 mm with deep S-wave, i.e., representing left ventricular force due to posteriorly displaced left ventricle (LV) by severely dilated RA and RV as tight mitral stenosis with severe pulmonary arterial hypertension and right ventricular dysfunction [Figure 1b]. 2D echo showed situs solitus levocardia with D-loop ventricle, pinpoint (3 mm in diameter), valvular pulmonary stenosis (both short axis and long axis), severely hypertrophied and dilated RV (6.3 cm), dilated giant RA (8.3 cm) versus left atrium (2.9 cm), and significant pericardial effusion (posteriorly in diastole 3 cm [Figure 2a]. Interatrial septum and interventricular septum (IVS) were intact. The dilated origin and proximal part of right coronary were seen distinctively with normal origin of left coronary artery. D-shaped LV with paradoxical IVS was characteristic. RV: LV ratio was 2.2:1. The thickness of RV free wall, IVS, and LV posterior wall by M-mode echo were 1.3, 2.3, and 1.1 cm, respectively. Poststenotic dilatation of main and branch pulmonary artery (PA) was obvious. There was no infundibular hypertrophy despite long-standing critical PVS with delayed presentation. There was significant tricuspid regurgitation, RV dysfunction (tricuspid annular peak systolic excursion [TAPSE]: 0.8 cm), and dilatation of inferior vena cava (3 cm with <25% respiratory variation and marked by traffic jam sign). Pulmonary valve (PV) was tricuspid, pliable, and doming with pinpoint stenosis [Figure 2b and c, Videos 1 and 2]. There was pan diastolic antegrade flow across PV [Figure 3a] because of significant rise of right ventricular end diastolic pressure. The right ventricular systolic pressure was 110 mmHg and PV gradient of 87 mmHg. Routine laboratory test showed hemoglobin of 8.8 g%, leukocyte count within normal range, and reversal of albumin: Globulin ratio. Other parameters such as urine routine examination, liver enzymes, serum bilirubin, and renal function test were normal. He was euthyroid. Peritoneal fluid and pericardial fluid analyses were transudate. Patient was treated with intravenous diuretic and dobutamine for 4 days before PBPV. A repeat 2D echo showed significant decrease in pericardial effusion, improvement in TASPE (1 cm), rise in PV gradient (130 mmHg), and PV jet velocity of 7 m/s [Figure 3b]. The diastolic anterograde flow across PV was seen only after atrial contraction [Figure 3b]. PV annulus was 2.4 cm. Two units packed cell was transfused overnight slowly with an additional diuretic dose. Hemoglobin improved to 11 g%. PBPV was done successfully using 24 mm × 40 mm ATLAS balloon BARD Peripheral Vascular, Inc. (BARD Canada, Inc.) through right femoral approach (sheath, 10 Fr). There was almost no residual gradient across the dilated PV [Video 3]. During procedure, the patient had one episode of nonsustained ventricular tachycardia (VT). The presence of 7 Fr Swan-Ganz catheter across the PV caused bradycardia and patient became transiently unresponsive during hemodynamic study. This hemodynamic instability improved by just pulling out the inflated balloon of wedge catheter across the PV because of mild improvement in forward flow. We watched for 30 min on catheterization table for any sign of flash pulmonary edema but there was none. The detail of periprocedural hemodynamic data of this is given in Table 1. Dobutamine was discontinued after 48 hours of procedure. A repeat echo was done after 4 days. Further improvement in pericardial effusion (1.1 cm), gradient across the PV (19 mmHg) [Figure 3c], TAPSE (1.6 cm), and diastolic function of RV (no antegrade filling of PA during any period of diastole) [Figure 3c] were remarkable. PV showed multiple irregular tears unlike pure commissural opening of mitral valvotomy [Figure 2d]. There was mild PV regurgitation. The echocardiographic changes were reduced right atrial size to 6.3 cm, left atrial size improved to 3.6 cm, and RV size improved to 4.5 cm × 2.6 cm. The complete resolution of pericardial effusion and normalization of albumin: Globulin ratio took 1 month. This patient returned to his routine life 3 months postprocedure.

Figure 1

(a) 12-lead electrocardiogram shows right ventricular hypertrophy with strain but no deep S-wave in V4–V6 leads; (b) V4–V6 leads in V7–V9 position showed diminished R-wave amplitude in posterior most leads representing left ventricle with deep S-wave

Figure 2

(a) Echocardiography shows right atrial enlargement, right ventricular hypertrophy with dilatation, small left ventricle, inconspicuously left atrium because of leftward shifted right atrial septum significant pericardial effusion without right atrium or right ventricle diastolic collapse due to higher diastolic pressure in these two chambers; (b) Parasternal short axis showed critically stenosis of tricuspid pulmonary valve in the annular plane; (c) Parasternal short axis showed doming and pliable pulmonary valve in the systole in the long axis of pulmonary artery; (d) Multiple irregular tears in the doming pulmonary valve quite apparent after percutaneous balloon pulmonary valvotomy

Figure 3

(a) Continous wave Doppler showed pan diastolic anterograde flow across pulmonary valve due to high right ventricular end diastolic pressure as was seen at the time of presentation; (b) After 4 days of dobutamine and diuretic infusion and blood transfusion, the anterograde diastolic flow across the pulmonary valve is limited to atrial contraction (correlates with tagged 12 lead electrocardiogram); (c) no more diastolic anterograde flow across the pulmonary valve after percutaneous balloon pulmonary valvotomy, some amount of pulmonary valve regurgitation and the gradient across the pulmonary valve is <20 mmHg after percutaneous balloon pulmonary valvotomy

Table 1

Periprocedural hemodynamic changes of critical valvular pulmonary stenosis with right ventricular dysfunction undergoing percutaneous balloon pulmonary valvotomy

Preprocedural evaluation

Clinical examination

At presentation: ESM-III/VI at presentation and almost inaudible ejection click

After 48 h of dobutamine and diuretic infusion: ESM increased to IV/VI with palpable pulmonary valve click

Echocardiography

Before dobutamine and diuretic infusion: PJV=4 m/s; pan diastolic anterograde flow across pulmonary valve, TAPSE=0.8 cm

After dobutamine and diuretic infusion: PJV=6 m/s; anterograde flow across pulmonary valve was only by atrial contraction; TAPSE=1 cm

Periprocedural invasive hemodynamic (Age: 39 year; sex: Male; height: 164 cm; weight: 44 kg; oxygen consumption: 126 ml/min; Hb%: 11 g%; HR: 84/min)

Before PBPV - RAP: 5 mmHg; RVP: 130/19 mmHg; PAP: 20/5/10 mmHg; PCWP: 5 mmHg; RFAP: 110/65 mmHg; LVP: 110/10 mmHg; RA SO2: 47%; PA saturation: 46%; FASO2: 95%; CO: 1.8 L/min

After PBPV - RAP: 2 mmHg; RVP: 47/6 mmHg; PAP: 40/6/15 mmHg; PCWP: 13 mmHg; RFAP: 150/80 mmHg; LVP: 140/13 mmHg; RA SO2: 57%; PA saturation: 58%; FA SO2: 96%; CO: 2.2 L/min

Post PBPV echo

RVSP: 31 mmHg; PAEDP: 8 mmHg; TAPSE: 1.6 cm; no anterograde diastolic flow across pulmonary valve; marked improvement in LV and LA size

ESM = Ejection systolic murmur, PJV = Pulmonary valve jet velocity, TAPSE = Tricuspid annular peak systolic excursion, RAP = Right atrial pressure, RVP = Right ventricular pressure, PAP = Pulmonary artery pressure, PCWP = Pulmonary capillary wedge pressure, RFAP = Right femoral arterial pressure, LVP = Left ventricular pressure, RA = Right atrium, PA = Pulmonary artery, FA = Femoral artery, CO = Cardiac output, RVSP = Right ventricular systolic pressure, PAEDP = Pulmonary artery end diastolic pressure, LV = Left ventricle, LA = Left atrium, SO2 = Oxygen saturation

DISCUSSION

The incidence of critical PS with delayed presentation with right heart failure but without cyanosis is rare.[1] Usual presentation is anasarca with or without cyanosis. The standard of care is PBPV after inotropic and diuretic for a few days. Dobutamine challenge dose tests the contractile reserve and improves systolic and diastolic functions similar to aortic stenosis with left ventricular dysfunction. Anasarca, pericardial effusion, and congestive hepatomegaly also improve. Percutaneous balloon valvotomy of critically stenosed pulmonary valve with right ventricular failure is very challenging. The procedure in such a situation may be associated with complications like balloon or wire induced ventricular tachycardia (VT), significant bradycardia during crossing the pulmonary valve, further increase of right ventricle afterload due to significant contraction of hypertrophied right ventricular outflow tract, rupture of pulmonary valve annulus and tamponade. Further worsening of right heart function, suicidal RV failure, the disclosure of severe infundibular stenosis, flash pulmonary edema, and infective endocarditis of PV may follow procedure. Flash pulmonary edema usually occurs within one hour of PBPV.[4] Rapid increase in pulmonary blood flow to an unprepared pulmonary circuit, relatively smaller size LV because of septal hypertrophy and its protrusion into LV, relatively noncompliant nature LV and associated pericardial effusion are the possible explanations for flash pulmonary edema. Rapid dilatation of PV followed removal of deflated balloon from PV prevents bradycardia, hypotension, and unconscious due low cerebral perfusion. We aligned the middle point of two markers in the ATLAS balloon across the annulus for proper dilatation of valve in one go because recrossing the valve with lost profile balloon is difficult during repeated dilatation which is detrimental for such sick patient. When it is difficult to align the waist of balloon across the pulmonary valve annulus, a tug of Amplatzer extra stiff (AES) wire and the rotation of the shaft of the balloon may help. Proper deflation of balloon after the dilatation of valve is essential for easy removal. The balloon size chosen was exactly 100% of PV annulus. The systolic PA pressure was around 30 mmHg up to 30 min after PBPV which explains why our patient had no pulmonary edema. An anecdotal case report shows use of Inoue balloon is safe for dilatation in cases where the crossing of RVOT is difficult because of infundibular stenosis in cases of long-standing critical pulmonary stenosis.[5] The graded dilatation of PS reduces chances of pulmonary edema.[6] In our case, we did not use incremental dilation up to 140% because of very good result from pliable valve without almost any infundibular stenosis. Sometimes, the resulted pulmonary edema from rapid increase in pulmonary blood flow and unprepared LV may need positive pressure ventilation for a week or so.[1] Hypotension is mostly due hyperactive infundibular stenosis can be tackled by infusion of short active beta-blocker-like esmolol.

The marked immediate improvement in systolic and diastolic function of RV after PBPV was remarkable in our case because of well-preserved right ventricular contractile reserve as evident by dobutamine challenge. The periprocedural hemodynamic improvement was documented by both invasive and echocardiographic evaluations as mentioned in Table 1.

CONCLUSION

RV has the unique ability to withstand very high pressure and volume overload for long duration. Critical valvular pulmonary stenosis with right heart failure without cyanosis is rare. Dobutamine stress echocardiography can be used as a useful tool to predict and monitor changes in systolic and diastolic function of RV around balloon pulmonary valvotomy. A pure pulmonary valvular stenosis in comparison to a combination of valvular and infundibular stenosis offers better result.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.