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Case Report

Case Report

Cavopulmonary Anastomosis in a Patient With Arrhythmogenic Right Ventricular Cardiomyopathy With Severe Right Ventricular Dysfunction

World Journal for Pediatric and Congenital Heart Surgery

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ª The Author(s) 2017 Reprints and permission:

Case Report Cavopulmonary Anastomosis in a Patient With Arrhythmogenic Right Ventricular Cardiomyopathy With Severe Right Ventricularsagepub.com/journalsPermissions.nav DOI: 10.1177/2150135117707459 journals.sagepub.com/home/pch Swaminathan Vaidyanathan, MBBS , Sivakumar Kothandam, DM , Rajesh Kumar, MD , Sujatha Desai Indrajith, DNB , and Ravi Agarwal, MCh Abstract A 26-year-old lady presented with exertional dyspnea, palpitations, central cyanosis, and oxygen saturations of 80% in room air. Her electrocardiogram, echocardiogram, and cardiac magnetic resonance were diagnostic of arrhythmogenic right ventricular dysplasia. There was no documented ventricular arrhythmia or syncopal episodes and Holter recordings were repeatedly normal. Cardiac hemodynamics showed right to left shunt through atrial septal defect, low pulmonary blood flow, normal atrial pressures, and minimally elevated right ventricular end-diastolic pressures. Since her presenting symptoms and cyanosis were attributed to reduced pulmonary blood flow, she underwent off-pump cavopulmonary anastomosis between right superior vena cava and right pulmonary artery. As we intended to avoid the adverse effect of extracorporeal circulation on the myocardial function and pulmonary vasculature, we did not attempt to reduce the size of the atrial septal defect. Her postoperative period was uneventful; oxygen saturation improved to 89% with significant improvement in effort tolerance. At 18-month follow-up, there were no ventricular arrhythmias on surveillance. The clinical presentation of this disease may vary from serious arrhythmias warranting defibrillators and electrical ablations at one end to right ventricular pump failure warranting cardiomyoplasty or right ventricular exclusion procedures at the other end. However, when the presentation was unusual with severe cyanosis through a stretched foramen ovale leading to reduced pulmonary blood flows, Glenn shunt served as a good palliation and should be considered as one of the options in such patients. Keywords arrhythmia, atrial septal defect (ASD), cardiomyopathy, cavopulmonary anastomosis, sudden cardiac death, magnetic resonance imaging (MRI), off pump surgery, ventricle, right, cyanosis Submitted January 02, 2017; Accepted April 06, 2017. Introduction Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited heart muscle disease character- ized by right ventricular (RV) dysfunction and ventricular arrhythmias. Unique feature of this disease is fibrofatty replacement of the RV myocardium. It is considered to be of genetic origin with autosomal dominant inheritance. It is usually diagnosed between 20 and 40 years of age and common presenting symptoms a re palpitations, syncope, or sudden cardiac death. We report a patient who had unusual presentation with central cyano sis and clubbing and an atrial septal defect (ASD) shunting right to left due to severe RV dysfunction simulating a cyanotic heart disease with reduced pulmonary blood flows. Case Report A 26-year-old female came to us with complaints of exertional dyspnea and palpitations. There were no documented ventricu- lar arrhythmias in past and on Holter study as well. On general Department of Cardiac Surgery, Madras Medical Mission, Chennai, India Department of Paediatric Cardiology, Madras Medical Mission, Chennai, India Department of Anesthesiology, Madras Medical Mission, Chennai, India Corresponding Author: Swaminathan Vaidyanathan, Depart ment of Cardiac Surgery, Madras Medical Mission, 4-A, Dr J Jayalalitha Nagar, Mogappair, Chennai 600037, Tamil Nadu, India. Email: swaminathan_27@yahoo.co.in " id="pdf-obj-0-21" src="pdf-obj-0-21.jpg">

Swaminathan Vaidyanathan, MBBS 1 , Sivakumar Kothandam, DM 2 , Rajesh Kumar, MD 2 , Sujatha Desai Indrajith, DNB 3 , and Ravi Agarwal, MCh 1

Abstract

A 26-year-old lady presented with exertional dyspnea, palpitations, central cyanosis, and oxygen saturations of 80% in room air. Her electrocardiogram, echocardiogram, and cardiac magnetic resonance were diagnostic of arrhythmogenic right ventricular dysplasia. There was no documented ventricular arrhythmia or syncopal episodes and Holter recordings were repeatedly normal. Cardiac hemodynamics showed right to left shunt through atrial septal defect, low pulmonary blood flow, normal atrial pressures, and minimally elevated right ventricular end-diastolic pressures. Since her presenting symptoms and cyanosis were attributed to reduced pulmonary blood flow, she underwent off-pump cavopulmonary anastomosis between right superior vena cava and right pulmonary artery. As we intended to avoid the adverse effect of extracorporeal circulation on the myocardial function and pulmonary vasculature, we did not attempt to reduce the size of the atrial septal defect. Her postoperative period was uneventful; oxygen saturation improved to 89% with significant improvement in effort tolerance. At 18-month follow-up, there were no ventricular arrhythmias on surveillance. The clinical presentation of this disease may vary from serious arrhythmias warranting defibrillators and electrical ablations at one end to right ventricular pump failure warranting cardiomyoplasty or right ventricular exclusion procedures at the other end. However, when the presentation was unusual with severe cyanosis through a stretched foramen ovale leading to reduced pulmonary blood flows, Glenn shunt served as a good palliation and should be considered as one of the options in such patients.

Keywords

arrhythmia, atrial septal defect (ASD), cardiomyopathy, cavopulmonary anastomosis, sudden cardiac death, magnetic resonance imaging (MRI), off pump surgery, ventricle, right, cyanosis

Submitted January 02, 2017; Accepted April 06, 2017.

Introduction

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited heart muscle disease character- ized by right ventricular (RV) dysfunction and ventricular arrhythmias. Unique feature of this disease is fibrofatty replacement of the RV myocardium. It is considered to be of genetic origin with autosomal dominant inheritance. It is usually diagnosed between 20 and 40 years of age 1 and common presenting symptoms a re palpitations, syncope, or sudden cardiac death. We report a patient who had unusual presentation with central cyano sis and clubbing and an atrial septal defect (ASD) shunting right to left due to severe RV dysfunction simulating a cyanotic heart disease with reduced pulmonary blood flows.

Case Report

A 26-year-old female came to us with complaints of exertional dyspnea and palpitations. There were no documented ventricu- lar arrhythmias in past and on Holter study as well. On general

1 Department of Cardiac Surgery, Madras Medical Mission, Chennai, India 2 Department of Paediatric Cardiology, Madras Medical Mission, Chennai, India 3 Department of Anesthesiology, Madras Medical Mission, Chennai, India

Corresponding Author:

Swaminathan Vaidyanathan, Depart ment of Cardiac Surgery, Madras Medical Mission, 4-A, Dr J Jayalalitha Nagar, Mogappair, Chennai 600037, Tamil Nadu, India. Email: swaminathan_27@yahoo.co.in

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World Journal for Pediatric and Congenital Heart Surgery XX(X)

Abbreviations and Acronyms

ARVC/D

arrhythmogenic right ventricular cardiomyo-

ASD

pathy/dysplasia atrial septal defect

CVP

central venous pressure

ECG

electrocardiogram

MPA

main pulmonary artery

MRI

magnetic resonance imaging

PA

pulmonary artery

RV

right ventricle

RA

right atrial

2 World Journal for Pediatric and Congenital Heart Surgery XX(X) Abbreviations and Acronyms ARVC/D arrhythmogenic right

Figure 1. Electrocardiogram showing epsilon wave.

2 World Journal for Pediatric and Congenital Heart Surgery XX(X) Abbreviations and Acronyms ARVC/D arrhythmogenic right

Figure 2. Transthoracic echocardiogram showing dilated RA, RV, and ASD. ASD indicates atrial septal defect; RA, right atrium; RV, right ventricle.

examination, she had central cyanosis and pan digital clubbing. Pulse oximeter reading was 80%. Her cardiovascular system examination showed cardiomegaly, wide split second heart sound, and right ventricular third heart sound. Her electrocar- diogram ([ECG] Figure 1) showed classical epsilon waves sug- gestive of ARVC/D. Echocardiogram (Figure 2) revealed moderate sized ASD, gross right atrial/RV (RA/RV) dilatation, severe RV systolic dysfunction, and anterior RV infundibular

2 World Journal for Pediatric and Congenital Heart Surgery XX(X) Abbreviations and Acronyms ARVC/D arrhythmogenic right

Figure 3. The RV angiogram with pigtail catheter in AP cranial view shows dilated RV with multiple coarse trabeculations. AP, Anterior- posterior; RV, right ventricular.

2 World Journal for Pediatric and Congenital Heart Surgery XX(X) Abbreviations and Acronyms ARVC/D arrhythmogenic right

Figure 4. The MRI: Arrow showing fibrofatty infiltration of right ventricle. MRI indicates magnetic resonance imaging.

wall bulgings. 2 There is no tricuspid and pulmonary valve abnormality. Cardiac catheterization showed RA pressure of 7 mm Hg, pulmonary artery (PA) pressure of 20/6 (11) mm Hg, low cardiac output of 1.3 L/min/m 2 , BSA and Pulmonary to Systemic flow ratio (Qp/Qs) was 0.85:1 due to right to left atrial shunt. The indexed pulmonary vascular resistance was 4.3 Wood units. The Pulmonary Vascular Resistance (PVR) was high as the Qp was very low. Right ventriculogram (Figure 3) showed dilated RV with multiple coarse transversely arranged trabeculations separated by deep fissures, poor con- tractility, and slow movement of the contrast to main pulmon- ary artery (MPA). Magnetic resonance imaging (MRI) showed (Figure 4) fibrofatty infiltration of RV. After multidisciplinary team meeting, she was planned for cavopulmonary anastomo- sis, to unload the RV and increase the pulmonary blood flow, since she was symptomatic with cyanosis and desaturation. At surgery, needle pressures were taken, aortic pressure was 105/ 78 (90) mm Hg versus PA pressure 24/15 (19) mm Hg, central

Vaidyanathan et al

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venous pressure (CVP) was 17 mm Hg. There was evidence of the literature. 8 Genetic analysis, one of the diagnostic criteria, fibrosis over the RV with good sized main and branch PAs. She was not carried out in the case we present here. 1 The manage- underwent off-pump cavopulm onary anastomosis between ment of ARVD still remains challenging and requires a flexible right superior vena cava and right PA. Her postoperative period approach, according to the presentation of the patient. Modified was uneventful and her saturation improved to 89 %. She Glenn shunt can be a good palliation in patients with severe remained in the intensive care unit for 2 days and was dis- cyanosis and reduced pulmonary blood flow. Since closure of charged home 1 week after the surgery. Patient is asympto- the ASD and completion of one-a nd-a-half ventricle repair matic at 18-month follow-up. Since there were no ventricular would have required extracorporeal circulation with its atten- arrhythmia on follow-up, implantation of cardioverter defibril- dant effects on myocardial and lung function, we did not opt for

lator was deferred.

it.

Discussion

Arrhythmogenic RV dysplasia (ARVD) leads to dilatation of RV with bulges located in the infundibular, apical, and subtri- cuspid areas (triangle of dysplasia). 3 Typical histological pat- tern of ARVD consists of replacement of midmural and external layers of RV myocardium by fatty tissue and fibrosis. The spectrum varies from typical arrhythmic forms to non- arrythmogenic forms which present with pump failure or severe cyanosis due to right to left atrial shunt. 4 An additional insult like viral myocarditis can lead to decrease in cardiac function and accelerate progression of the disease. 1 The revised task force criteria were based on structural (echocardiography/ MRI/RV angiography), histological, ECG, arrhythmic and familial features of the disease. 1 The typical presentations are palpitations, fatigue, or syncope. Some may present as sudden cardiac death. Postmortem examinations after sudden death in young patients under 35 years showed evidence of ARVD in 20% of patients. 5 There are rare reports of patients presenting with marked right to left shunt, cyanosis, and cerebral abscess without any arrhythmia. 6 Our patient also had cyanosis and clubbing without any history of ventricular arrhythmias. Treatment options for serious arrhythmias include electrical ablations and implantable cardioverter defibrillator. Complete surgical disarticulation of RV is reserved for refractory patients. 7 Since our patient did not have any symptoms or signs of ventricular tachycardia, she was managed conservatively with beta blockers and had been counseled about the need for defibrillator implant in the future. If the presentation is primar- ily with pump failure, RV exclusion surgery, cardiomyoplasty, or heart transplantation may be the only feasible option. In addition to resolving arrhythmia and RV failure, RV exclusion surgery can improve LV function by decreasing paradoxical interventricular septal moti on. Our patient also underwent off-pump bidirectional modified Glenn shunt to unload the dysfunctional RV which improved her cyanosis/exercise capacity, a similar strategy adopted in one previous report in

Authors’ Note

Permission was obtained from the subject patient to publish this case report.

Declaration of Conflicting Interests

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding

The author(s) received no financial support for the research, author- ship, and/or publication of this article.

References

  • 1. Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis of arrhyth- mogenic right ventricular cardiomyopathy/dysplasia. Eur Heart J. 2010;19(7): 25.

  • 2. Daliento L, Rizzoli G, Thiene G, et al. Diagnostic accuracy of right ventriculography in arrhythmogenic right ventricular cardiomyo- pathy. Am J Cardiol. 1990;66(7): 741-745.

  • 3. Marcus FI, Fontaine GH, Guiraudon G, et al. Right ventricular dysplasia: a report of 24 adult cases. Circulation. 1982;65(2): 384-398.

  • 4. Fontaine G, Fontaliran F, Frank R. Arrhythmogenic right ventri- cular cardiomyopathies clinical forms and main differential diag- noses. Circulation. 1998;97(16): 1532-1535.

  • 5. Thiene G, Nava A, Corrado D, Rossi L, Pennelli N. Right ventri- cular cardiomyopathy and sudden death in young people. N Engl J Med. 1988;318(3): 129-133.

  • 6. Matsuoka Y, Kawaguchi K, Okishima T, Hayakawa K. An infant with suspected right ventricular dysplasia presenting unique ventriculograms. Clin Cardiol. 1988;11(1): 55-58.

  • 7. Michael KA, Morgan JM. Not letting the left side know what the right is doing! Eur J Cardiothorac Surg. 2008;33(4): 748-750.

  • 8. Gorgulu S, Nurkalem Z, Celebi A, et al. Unusual presentation of a patient with arrhythmogenic right ventricular dysplasia treated with a Glenn shunt. Int J Cardiol. 2006;113(3): 410-413.