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Case Report

World Journal for Pediatric and

Congenital Heart Surgery
Cavopulmonary Anastomosis in a 1-3
The Author(s) 2017
Reprints and permission:
Patient With Arrhythmogenic Right sagepub.com/journalsPermissions.nav
DOI: 10.1177/2150135117707459
Ventricular Cardiomyopathy With journals.sagepub.com/home/pch

Severe Right Ventricular Dysfunction

Swaminathan Vaidyanathan, MBBS1, Sivakumar Kothandam, DM2,

Rajesh Kumar, MD2, Sujatha Desai Indrajith, DNB3,
and Ravi Agarwal, MCh1

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.

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 Case Report

Arrhythmogenic right ventricular cardiomyopathy/dysplasia A 26-year-old female came to us with complaints of exertional
(ARVC/D) is an inherited heart muscle disease character- dyspnea and palpitations. There were no documented ventricu-
ized by right ventricular (RV) dysfunction and ventricular lar arrhythmias in past and on Holter study as well. On general
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 1
Department of Cardiac Surgery, Madras Medical Mission, Chennai, India
usually diagnosed between 20 and 40 years of age1 and 2
Department of Paediatric Cardiology, Madras Medical Mission, Chennai, India
common presenting symptoms are palpitations, syncope, or Department of Anesthesiology, Madras Medical Mission, Chennai, India
sudden cardiac death. We report a patient who had unusual
presentation with central cyanosis and clubbing and an atrial Corresponding Author:
Swaminathan Vaidyanathan, Department of Cardiac Surgery, Madras
septal defect (ASD) shunting right to left due to severe RV Medical Mission, 4-A, Dr J Jayalalitha Nagar, Mogappair, Chennai 600037,
dysfunction simulating a cyanotic heart disease with Tamil Nadu, India.
reduced pulmonary blood flows. Email: swaminathan_27@yahoo.co.in
2 World Journal for Pediatric and Congenital Heart Surgery XX(X)

Abbreviations and Acronyms

ARVC/D arrhythmogenic right ventricular cardiomyo-
ASD 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

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.

Figure 1. Electrocardiogram showing epsilon wave.

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/m2, 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
Figure 2. Transthoracic echocardiogram showing dilated RA, RV, and 4.3 Wood units. The Pulmonary Vascular Resistance (PVR)
ASD. ASD indicates atrial septal defect; RA, right atrium; RV, right 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-
examination, she had central cyanosis and pan digital clubbing. tractility, and slow movement of the contrast to main pulmon-
Pulse oximeter reading was 80%. Her cardiovascular system ary artery (MPA). Magnetic resonance imaging (MRI) showed
examination showed cardiomegaly, wide split second heart (Figure 4) fibrofatty infiltration of RV. After multidisciplinary
sound, and right ventricular third heart sound. Her electrocar- team meeting, she was planned for cavopulmonary anastomo-
diogram ([ECG] Figure 1) showed classical epsilon waves sug- sis, to unload the RV and increase the pulmonary blood flow,
gestive of ARVC/D. Echocardiogram (Figure 2) revealed since she was symptomatic with cyanosis and desaturation. At
moderate sized ASD, gross right atrial/RV (RA/RV) dilatation, surgery, needle pressures were taken, aortic pressure was 105/
severe RV systolic dysfunction, and anterior RV infundibular 78 (90) mm Hg versus PA pressure 24/15 (19) mm Hg, central
Vaidyanathan et al 3

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 cavopulmonary 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-and-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 Authors Note

Permission was obtained from the subject patient to publish this case
Arrhythmogenic RV dysplasia (ARVD) leads to dilatation of report.
RV with bulges located in the infundibular, apical, and subtri-
cuspid areas (triangle of dysplasia).3 Typical histological pat-
Declaration of Conflicting Interests
tern of ARVD consists of replacement of midmural and
external layers of RV myocardium by fatty tissue and fibrosis. The author(s) declared no potential conflicts of interest with respect to
the research, authorship, and/or publication of this article.
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 Funding
insult like viral myocarditis can lead to decrease in cardiac The author(s) received no financial support for the research, author-
function and accelerate progression of the disease.1 The revised ship, and/or publication of this article.
task force criteria were based on structural (echocardiography/
MRI/RV angiography), histological, ECG, arrhythmic and References
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