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

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 Case Report

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 age1 and
common presenting symptoms are palpitations, syncope, or
sudden cardiac death. We report a patient who had unusual
presentation with central cyanosis 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.
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, Department of Cardiac Surgery, Madras
Medical Mission, 4-A, Dr J Jayalalitha Nagar, Mogappair, Chennai 600037,
Tamil Nadu, India.
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-
pathy/dysplasia
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

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
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
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
venous pressure (CVP) was 17 mm Hg. There was evidence of
fibrosis over the RV with good sized main and branch PAs. She
underwent off-pump cavopulmonary anastomosis between
right superior vena cava and right PA. Her postoperative period
was uneventful and her saturation improved to 89%. She
remained in the intensive care unit for 2 days and was dis-
charged home 1 week after the surgery. Patient is asympto-
matic at 18-month follow-up. Since there were no ventricular
arrhythmia on follow-up, implantation of cardioverter defibril-
lator was deferred.
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 motion. 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
3
the literature.8 Genetic analysis, one of the diagnostic criteria,
was not carried out in the case we present here.1 The manage-
ment of ARVD still remains challenging and requires a flexible
approach, according to the presentation of the patient. Modified
Glenn shunt can be a good palliation in patients with severe
cyanosis and reduced pulmonary blood flow. Since closure of
the ASD and completion of one-and-a-half ventricle repair
would have required extracorporeal circulation with its atten-
dant effects on myocardial and lung function, we did not opt for
it.
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.
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