dr. priyadarshini. a

70 Pages·2016·2.92 MB·English

SHORT AND INTERMEDIATE TERM FOLLOW UP OF ALL
INFANTS DIAGNOSED WITH PULMONARY ATRESIA AND
VENTRICULAR SEPTAL DEFECT - A FIVE YEAR FOLLOW UP
STUDY

PROJECT REPORT
Submitted during the course of DM Cardiology by
DR. PRIYADARSHINI. A
Trainee
DEPARTMENT OF CARDIOLOGY
Jan 2014 – Dec 2016

SREE CHITRA TIRUNAL INSTITUTE FOR MEDICAL
SCIENCES AND TECHNOLOGY, TRIVANDRUM, KERALA,
DECLARATION

I, Dr. Priyadarshini A, hereby declare that the project in this book, titled
“Short and intermediate term follow up of all infants diagnosed with pulmonary
atresia and ventricular septal defect - A five year follow up study” was
undertaken by me under the supervision of the faculty, Department of
Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology.

Trivandrum Dr Priyadarshini A
3/10/16 DM Cardiology trainee

FORWARDED
The candidate, Priyadarshini A, has carried out the minimum required
project.

Thiruvananthapuram Prof. (Dr )Ajit Kumar VK
3/10/16 Head of Department of Cardiology
CERTIFICATE

This is to certify that this thesis titled “Short and intermediate term follow up of
all infants diagnosed with pulmonary atresia and ventricular septal defect - a
five year follow up study” has been prepared by Dr. Priyadarshini. A, DM
Cardiology Resident, Department of Cardiology at Sree Chitra Tirunal Institute
for Medical Sciences & Technology, Thiruvananthapuram. She has shown keen
interest in preparing this project.

GUIDE
Dr. Krishnamoorthy.K.M
Professor, Department of Cardiology
SCTIMST, Thiruvananthapuram

CO GUIDE
Dr. Deepa S. Kumar
Assistant Professor, Department of Cardiology
SCTIMST, Thiruvananthapuram
Dedicated to my
teachers, family and
friends
TABLE OF CONTENTS

Abstract i
Introduction 1
Aims and Objectives 6
Review of literature 8
Materials and methods 17
Statistical analysis 19
Results 20
Discussion 42
Conclusion 48
Limitation 49
References 50
Annexure 54
ABBREVIATIONS

VSD – Ventricular septal defect
PA – Pulmonary atresia
MAPCA – Major Aorto-pulmonary collaterals
PDA - Patent ductus arteriosus
RVOT – Right ventricular outflow tract
CT – Computed tomography
MRI – Magnetic resonance imaging
TOF – Tetralogy of Fallot
DRPA – Diameter of right pulmonary artery at the hilum
DLPA – Diameter of left pulmonary artery at the hilum
DDTAO- Diameter of descending thoracic Aorta at the diaphragm
ABSTRACT:
Introduction: Ventricular septal defect- pulmonary atresia (VSD-PA)
encompasses a spectrum of anomalies ranging from short to long segment
pulmonary atresia and duct dependent or MAPCA dependent pulmonary
circulation or a combination of both. From the therapeutic standpoint, surgical
treatment could vary from as simple as VSD closure with RVOT reconstruction
to as complicated as multi-staged unifocalisation. More over children may
require interim palliative procedures in the form of ductal stenting, Aorto-
pulmonary shunt or Cavo-pulmonary anastomosis. There is paucity of data from
the Indian subcontinent regarding the outcomes in this cohort of patients.
Aim: To study variations in pulmonary anatomy, natural history, time to
intervention, complications, outcomes and determinants of mortality in infants
with VSD-PA.
Methodology: All infants (<1 year) registered at our institute between January
2011 and December 2015 with the diagnosis of VSD-PA were enrolled.
Children with complex anatomy like Single ventricle, transposition, AV canal
defects etc were excluded from the study. Deaths were classified according to
the time period in which attrition occurred, T1- before any palliation (Aorto-
pulmonary shunt, PDA stenting, BDG)/ definitive surgery was done, T2- in the

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interstage between palliation and definitive repair, T3- postoperative period and
T4- late after definitive repair
Results: A total of 108 infants were included in the study. There was an almost
equal gender distribution- 51% were male and 49% were female. Mean age at
presentation was 1.65 months (range 0 – 11 months) and mean duration of
follow up was 18.9 months. 73% had confluent pulmonary anatomy and long
segment pulmonary atresia was more common. Majority of patients (55.6%)
had a ductal dependence, 25.9% had MAPCA dependence and 18.5% had a
combination of both. Right arch was seen in 25.9 % of the children. Mean Mc
Goon index was 1.26. 7 patients were diagnosed with Di George syndrome. The
average birth weight was 2.64 kg and 12 % were born preterm. There was
history of maternal diabetes in 17%. The mean lowest saturation on follow up
was 73.4 %. 32.4% required prostaglandin support. Imaging in the form of CT/
MR was required in 52% of the population. Imaging was very useful in
MAPCA dependent pulmonary circulation to plan surgery. 6 required ductal
stenting, 37 required Aorto-pulmonary shunt and 4 required BDG. Average age
at initial palliation was 6.24 months. Average time to palliation after diagnosis
was 4.81 months. Out of the 43 patients, who underwent palliative procedures,
13 (29%) proceeded to definitive repair. There were 32 deaths in toto – 19 in
the T1 period. 2 children were lost in the inter-stage period- T2. ICR was done
in 13 patients with the following concomitant procedures- homograft repair was

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done with intracardiac repair in 4 patients, 7 children did not require conduit -
only RVOT reconstruction sufficed and primary unifocalisation with intra
cardiac repair was done in 3 children. 10 children died in the post operative
period and one death was late- 6 months after surgery. Branch PA plasty was
concomitantly done in majority (69.2%) of the patients. Pulmonary anatomy-
length of atresia, was statistically significant as a predictor of mortality
(p=0.023). Survival at 1, 2 and 4 years were 70.3%, 68.2% and 65%
respectively.
Conclusion: Despite early diagnosis, mortality associated with VSD-PA
remains high with most of the deaths occurring in the T1 period, before any
palliation or corrective surgery is done. Surveillance and weekly saturation
monitoring may avert the catastrophes associated with worsening hypoxemia.
Keywords: Ventricular septal defect- pulmonary atresia, natural history,
outcomes, predictors of mortality.

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INTRODUCTION :
Pulmonary atresia with ventricular septal defect is a rare congenital
anomaly, constitutes about 2% of congenital heart diseases , is considered to be the
most severe form of Tetralogy of Fallot and occurs at a frequency of 0.07 per 1,000
live births ( Baltimore Washington study)(1). Anatomically, the right ventricular
outflow tract ends blindly, right ventricular stroke volume is directed towards the
Aorta through a large ventricular septal defect. Pulmonary arterial architecture is
complex, ranging from duct dependent pulmonary circulation to a pulmonary
arterial tree entirely fed by major Aorto-pulmonary collaterals (MAPCAs).
.VSD-PA can be classified into 3 types based on source of pulmonary blood flow.
A classification (2) has been proposed by the Congenital Heart Surgeons Society
(CHSS) based on pulmonary blood flow and is detailed below:
Type A: Native PAs present, pulmonary vascular supply through PDA and no APC
/ MAPCAs
Type B: Native PAs and APC/MAPCAs present
Type C: No native PAs, pulmonary blood supply through APC/MAPCAs only.

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