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Table of Contents
CASE REPORT
Year : 2022  |  Volume : 12  |  Issue : 3  |  Page : 139-142

Challenges in balloon pulmonary valvotomy for severe pulmonary stenosis presenting with cyanosis and ventricular dysfunction


1 Department of Pediatric Cardiology, Medica Super Specialty Hospital, Kolkata, West Bengal, India
2 Cardiac Anesthesia and Critical Care, Medica Super Specialty Hospital, Kolkata, West Bengal, India
3 Cardiac Anaesthesia and Critical Care Medica Super Specialty Hospital, Kolkata, West Bengal, India
4 Cardiology, Medica Super Specialty Hospital, Kolkata, West Bengal, India

Date of Submission31-Jul-2021
Date of Decision27-Sep-2021
Date of Acceptance27-Sep-2021
Date of Web Publication14-Sep-2022

Correspondence Address:
Dr. Anil Kumar Singhi
Senior Consultant and Head, Department of Pediatric and Congenital Heart Disease Medica Superspeciality Hospital, 127, Mukundapur, E.M Bypass, Kolkata - 700099, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jicc.jicc_46_21

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  Abstract 


Valvular pulmonary stenosis (PS) can present with cyanosis and right heart failure in older children and adults. Transcatheter balloon pulmonary valvuloplasty is a standard intervention for severe valvular PS in all age groups. Balloon dilatation in critical PS like presentation in older age groups has unique challenges in management. These cases are associated with right ventricular dysfunction, cyanosis, pulmonary edema, injury to right ventricular structure, and arrhythmia. Imaging is an essential component for diagnosis and management. We present two such cases of older children who presented with cyanosis and right ventricular dysfunction. Detailed imaging and team approach of care helped in managing the challenges of balloon pulmonary valvuloplasty in older sick substrates.

Keywords: Balloon valvuloplasty, cyanosis, imaging, pulmonary stenosis


How to cite this article:
Singhi AK, Mohapatra SK, Dey S, Chatterjee D, De A. Challenges in balloon pulmonary valvotomy for severe pulmonary stenosis presenting with cyanosis and ventricular dysfunction. J Indian coll cardiol 2022;12:139-42

How to cite this URL:
Singhi AK, Mohapatra SK, Dey S, Chatterjee D, De A. Challenges in balloon pulmonary valvotomy for severe pulmonary stenosis presenting with cyanosis and ventricular dysfunction. J Indian coll cardiol [serial online] 2022 [cited 2022 Oct 1];12:139-42. Available from: https://www.joicc.org/text.asp?2022/12/3/139/356066




  Introduction Top


Valvular pulmonary stenosis (PS) is one of the common types of congenital heart disease comprising 8% of congenital heart disease.[1] The presentation ranges from incidental detection of murmur in an asymptomatic patient to dyspnea on exertion, fatigability, or cyanosis in critical obstruction of the newborn. balloon pulmonary valvotomy (BPV) is the intervention of choice for significant valvular PS across all ages. Older children with severe valvular PS presenting like critical stenosis of the newborn have their own challenges during balloon pulmonary valvotomy. We report the management of two older children with severe valvular PS helped by appropriate imaging.


  Case Report Top


Case 1

A 12-year-old adolescent boy weighing 30 kg presented with effort intolerance (functional Class III) and exertional palpitation. He was cyanosed with oxygen saturation of 72% (SpO2) and clubbing. His neck veins were prominent along with abdominal distension. Cardiac evaluation revealed a single second heart sound with a very short systolic murmur in the second left intercostal border. X-ray of the chest showed mesocardia, cardiothoracic ratio of 0.5, and parahilar congestion. Electrocardiogram showed sinus rhythm, right axis deviation, and right ventricular hypertrophy with strain pattern. The echocardiography showed severely hypertrophied right ventricle (RV) with suprasystemic pressure and dysfunction. The pulmonary valve was doming with minimal antegrade flow, and the gradient across the pulmonary valve was more than 150 mmHg. The pulmonary valve annulus measured 14 mm. The patent foramen ovale was seen shunting right to left [[Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d and Movie 1]. The left ventricular (LV) systolic function was mildly reduced with mild pericardial effusion and systemic venous congestion. His basal hemoglobin was 21.2 gm/dl. He was taken for balloon pulmonary valvuloplasty. The right ventricular systolic pressure was 217 mmHg. The gradient across the pulmonary valve was 200 mmHg [Figure 2]. The pulmonary valve was crossed with the help of 4F Judkins right catheter and straight-tipped Terumo wire combination. The basal angiogram of the right ventricular outflow tract showed severe valvular PS with doming pulmonary valve and pinhole type opening [Figure 3]a. Balloon pulmonary valvotomy was done initially with 14 X 40mm Tyshak II balloon (NuMED Inc, NY) followed by 18 X 40 mm Tyshak II balloon. The final pullback gradient from pulmonary artery to RV came down to 50 mmHg with adequate leaflet excursion of pulmonary valve [[Figure 3]b and [Figure 3]c and Movie 2]. The oxygen saturation( SpO2) improved on table from 72% to 97 percent [Figure 2]. The patient tolerated the procedure well and was shifted to the intensive care unit (ICU) for observation. After 6 h, he developed significant desaturation (SpO2 65%) with hypotension. His echocardiographic evaluation showed significant infundibular narrowing of the right ventricular outflow tract (clamping of the infundibulum) with severe right ventricular dysfunction [[Figure 4]a and [Figure 4]d and Movie 3]. He was resuscitated with fluid and inotropic support. Beta-blocker was started for the infundibular component; bronchodilators and corticosteroid inhalation were given for reactive airway disease and noninvasive ventilatory support provided. He improved gradually with SpO2 96% on room air over the next 5 days. The right ventricular function and infundibular flow improved significantly over time [[Figure 4]b,[Figure 4]c, [Figure 4]d, [Figure 4]e, [Figure 4]f and Movie 3]. He was discharged in clinically stable condition after 7 days on beta-blocker and inhalers. He was doing well in the 3-month follow-up, and echocardiography revealed good pulmonary valve leaflet opening with mild gradient across improved right ventricular function with patent foramen ovale shunting predominantly left to right [[Figure 1]e, [Figure 1]f, [Figure 1]g, [Figure 1]h and Movie 1].
Figure 1: Transthoracic echocardiogram of first patient on admission in (a) parasternal short axis view showing Right ventricular hypertrophy supra systemic right ventricular pressure and RV dysfunction. (b) patent foramen ovale (PFO) showing right to left shunt ( White arrow) , (c ) parasternal view showing a severely narrowed pulmonary valve with severe trans valvular gradient (d). Three month post balloon pulmonary valvotomy(e) echocardiogram in parasternal short axis view right ventricular pressure became sub systemic with adequate function, (f)the PFO shunting left to right - arrow( g) and good flow across the pulmonary valve and (h)mild gradient

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Figure 2: (a) Basal suprasystemic right ventricular pressure, (b) low pulmonary artery pressure, and (c) low oxygen saturation. Post balloon valvotomy, the right ventricular systolic pressure significantly reduced (d) with corresponding mild elevation of the pulmonary artery pressure (e) and increase in oxygen saturation (f)

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Figure 3: Angiogram of first patient in basal state in right anterior view (a) showing doming pulmonary valve with pinhole opening (white arrow), Post balloon valvotomy angiogram in (b) right anterior view and (c) lateral view showing well open pulmonary valve with good flow (yellow arrow)

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Figure 4: Transthoracic echocardiogram of the first patient during intensive care unit stay in apical four-chamber view (a-c) and parasternal long-axis view (d-f) on different post procedure days. On the procedure day, echocardiogram (a and d) showed severe right ventricular dysfunction and infundibular clamping (arrow) which progressively improved on day 3 (b and e) and on day 5 (c and f)

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Case 2

A 6-year and 6-month-old boy weighing 21 kg presented with breathlessness on exertion with progressive cyanosis. He had an oxygen saturation of 79% along with clubbing. The second heart sound was single with Grade II/VI ejection systolic murmur in the upper left sternal border. The chest X-ray showed a cardiothoracic ratio of 0.5. His electrocardiogram had sinus rhythm, right axis deviation with right ventricular hypertrophy, and RV strain pattern. The echocardiogram showed very severe valvular PS, doming pulmonary valve with gradient across pulmonary valve more than 100 mmHg, and minimal flow across pulmonary valve. The pulmonary annulus was 13 mm. The patent foramen ovale was a tunnel like with right-to-left shunt. The ventricular systolic function was reduced. He was taken for balloon pulmonary valvuloplasty. Basal hemodynamic study revealed a gradient of 113 mmHg across the pulmonary valve. The pulmonary valve was dilated with a TYSHAK II balloon (NuMED Inc., NY, USA), 14 mm × 40 mm followed by a 16 mm × 40 mm balloon. The final pullback gradient across the pulmonary valve was 14 mmHg, good leaflet excursion. The SpO2 improved from 79% to 90%. The desaturation was attributed with persistent infundibular component and right ventricular stiffness resulting in right-to-left shunting across the atrial-level shunt. He was started on beta-blocker to alleviate infundibular stiffness. He was discharged in a clinically stable position on the 3rd day. On short-term follow-up, he is asymptomatic with oxygen saturation of 85%. Post procedure, echocardiogram showed good opening of pulmonary valve leaflet with gradient of 17 mmHg. The patent foramen ovale was shunting bidirectionally. The right ventricular systolic function was adequate. The left ventricle was dominant with adequate ventricular systolic function (LV ejection fraction of 55%).


  Discussion Top


Valvular PS is one of the common congenital heart defects. The relatively benign lesion usually presents with murmur and mild effort intolerance. The lesion is slowly progressive. Transcatheter balloon dilatation of pulmonary valve is the most accepted safe and efficacious intervention for valvular PS for all ages since its introduction three decades ago.[2] In the newborn period, it can present as a critical obstruction, cyanosis, and right ventricular dysfunction requiring urgent intervention. In older children and adults rarely in an undiagnosed or neglected case, the PS can progress and present with cyanosis and RV dysfunction like critical PS of the newborn. The rare complicated critical presentation in older children poses a challenge in management. They are usually sick substrates requiring stabilization in ICU in a team approach. The imaging of the lesion comes in the center point not only for diagnosis but also in the management of the patient during peri procedure and post procedure time. During procedure critical PS dilatation, the patient can develop suicidal RV, pulmonary edema, worsening right ventricular failure, ventricular arrhythmia, and trauma to the RV outflow tract.[3] Sudden relieving valvular obstruction can lead to the suicidal RV and clamping of the infundibulum resulting in significantly reduced pulmonary blood flow. The increasing cyanosis and RV dysfunction is a challenge in ICU management requiring inotropic support, ventilation, and beta-blocker therapy as required in our first case.[4] Frequent assessment of the cardiac status and imaging of the heart helped is to titrate the therapy appropriately. The accompanying infundibular stenosis can get unmasked after balloon dilatation of the pulmonary valve as in our series of two patients. This can result in persistent gradients. The right ventricular stiffness and residual infundibular stenosis can cause right-to-left shunting at the atrial-level communication and persistent cyanosis. Infundibular hypertrophy in long-standing cases of PS is a known entity which regresses gradually helped by beta-blocker within weeks to months.[5] In our series of two patients, detailed echocardiographic evaluation helped to manage the patients based on the hemodynamic status. In long standing severe pulmonary stenosis balloon pulmonary valvotomy may cause pulmonary edema. There are multiple contributing factors for this phenomenon. Sudden filling of lung fields due to opening of valve leaflets accompanied by small underfilled noncompliant left ventricle along with bowing of interventricular septum precipitates lung edema. Reperfusion injuries in long-standing cases have been reported up to 71%.[6] Fortunately, we had not encountered the same in our series. Some units prefer elective ventilation and graded dilatation for prevention of post BPV pulmonary edema. Usually, most of the patients have the persistence of long-standing ventricular dysfunction. Preprocedural milrinone or dobutamine has been used in some institutions for improving systolic and diastolic ventricular functions, thereby reducing the periprocedural complications. The beta-receptor antagonists and diuretics in addition to afterload-reducing agents would improve the function.[1],[6] Other challenges during dealing with such sick patients are periprocedural arrhythmias which may manifest during catheter manipulation or due to dilated right atrium and hypertrophy of RV. However, they respond well to anti-arrhythmic medications. Thromboembolism is another such complication augmented by RV dysfunction and the presence of right-to-left shunt which may lead to stroke-like episodes requiring anticoagulants. Manipulation of balloon across the RV outflow tract is known to cause multiple complications like vascular injuries, complete heart block and traumatic valvular hematoma. In view of possible complications a team of cardiac surgeons, cardiac anaesthetist and electrophysiologist is required as backup for any untoward incident. These complications need to be kept in my mind while performing the procedure. But most importantly, in spite of ticking all boxes in the checklist, the outcome can be unpredictable. The team managing such a sick subset of patients should have imaging facilities available bedside and experts should be able to analyze the echocardiographic images tailoring to the clinical status.


  Conclusion Top


Very severe valvular PS can present like critical PS with RV dysfunction and cyanosis in delayed scenarios. Balloon pulmonary valvuloplasty is the modality of choice for symptomatic older children and adolescents with severe pulmonary stenosis associated with challenges arising from long standing uncorrected heart disease. Detailed imaging is the key for the diagnosis and for on-site management of such patients in ICU. A well-informed and prepared team of experts consisting of interventional cardiologist, anesthesiologist, intensive care specialist, electrophysiologist, and backup cardiac surgeon can handle this critical obstruction of pulmonary outflow in favor of good outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients' parents have given their consent for images and other clinical information to be reported in the journal. The patients' parents understand that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Cuypers JA, Witsenburg M, van der Linde D, Roos-Hesselink JW. Pulmonary stenosis: Update on diagnosis and therapeutic options. Heart 2013;99:339-47.  Back to cited text no. 1
    
2.
Stanger P, Cassidy SC, Girod DA, Kan JS, Lababidi Z, Shapiro SR. Balloon pulmonary valvuloplasty: Results of the valvuloplasty and angioplasty of congenital anomalies registry. Am J Cardiol 1990;65:775-83.  Back to cited text no. 2
    
3.
Barik R, Akula SP, Damera SR. Use of dobutamine stress echocardiography for periprocedural evaluation of a case of critical valvular pulmonary stenosis with delayed presentation. J Cardiovasc Echogr 2016;26:56-60.  Back to cited text no. 3
    
4.
Al Kasab S, Ribeiro P, Al Zaibag M. Use of a double balloon technique for percutaneous balloon pulmonary valvotomy in adults. Br Heart J 1987;58:136-41.  Back to cited text no. 4
    
5.
Fontes VF, Esteves CA, Sousa JE, Silva MV, Bembom MC. Regression of infundibular hypertrophy after pulmonary valvuloplasty for pulmonic stenosis. Am J Cardiol 1988;62:977-9.  Back to cited text no. 5
    
6.
Tefera E, Qureshi SA, Bermudez-Cañete R, Rubio L. Percutaneous balloon dilation of severe pulmonary valve stenosis in patients with cyanosis and congestive heart failure. Catheter Cardiovasc Interv 2014;84:E7-15.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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