Pattern of Congenital Heart Diseases and Treatment Options in a Bangladeshi Center

Analysis of 6914 cases from Non-invasive Cardiac Laboratory

Prof. Brig. Gen. Nurun Nahar Fatema Begum Paediatric Interventional Cardiologist & Head of Department Department of Paediatrics, AFMC, CMH DHAKA

Introduction

The incidence of congenital heart disease is 25 per thousand live births showed in a study conducted in Bangladesh.
Echocardiography is the most sensitive, specific and cost effective means to diagnose congenital heart disease safely and correctly.
Echocardiography laboratory can give a scenario of pattern of congenital heart diseases existing in Bangladesh as well as natural history of cases and treatment options offered.
ECHO can be repeated as many times as required to see the progress, outcome and complications of treatment.

Objective

To see the pattern of congenital heart disease and available treatment facility in a cardiac center of Bangladesh.

Methods and materials

Types of study :	Hospital based retrospective observational study
Duration :	January to December 2012
Place of study :	LABAID cardiac center, Bangladesh
Sample size :	6914
Materials used :	Chest X-ray, ECG, Echocardiography,

Methods

Patients were referred from almost every district and remote areas of Bangladesh and they represent whole country scenario

Patients in LABAID cardiac center

Patients were waiting before cardiac catheterization in Cath Lab

Results

AGE DISTRIBUTION OF PATIENT (n= 6914)

SEX DISTRIBUTION OF PATIENT (n= 6914)

Distribution of abnormal & normal patient (n= 6914)

A. Acyanotic heart disease

Acyanotic Congenital Heart disease	Number	%
1. Ventricular Septal Defect( VSD)	1286	18.60
2. Atrial Septal Defects (ASD )	1066	15.41
3. Patent ductus arteriosus (PDA)	594	8.59
4. ASD,VSD, and PDA	482	6.97
5. Patent foramen ovale	295	3.83
6. Pulmonary stenosis PS	178	2.57
7. AtrioventricularSeptal defect (AVSD)	136	1.97
8. Aortic Stenosis (AS)	108	1.56
9. Branch pulmonary artery stenosis	65	0 .94
10, Coarctation of Aorta COA	60	0.87

11.Dilated cardiomyopathy DCM	56	0.81
12.Mitral valve prolapsed	42	0.61
13.Idiopathic pulmonary hypertension (IPAH)	31	0.45
14.Hypertrophic cardiomyopathy (HCM)	23	0.33
15. Restrictive cardiomyopathy	11	0.09
16. Anomalous origin of Left coronary artery from pulmonary artery( ALCAPA)	6	0.04
17.Mital stenosis	6	0.04
18. Others (AR, MR, TR, PR, Thrombus, vegetation, Tumor’s, aneurysms, carditis etc.)	389	5.19

B. Cyanotic Congenital Heart disease

B. Cyanotic Congenital Heart disease	Number	%
1.Tetralogy of Fallot (TOF)	331	4.79
2.Trans position of great Arteries (TGA)	156	2.26
3.Pulmonary Atresia	108	1.56
4.Doulde out let right ventricle (DORV)	94	1.36
5.Persistent pulmonary hypertension of newborn (PPHN)	73	1.06
6.Total anomalous pulmonary venous drainage (TAPVD)	66	0.95
7. Tricuspid atresia	62	0.89

8.Double Inlet left ventricule (DILV)	49	0.71
9.Complex congenital heart disease	32	0.46
10.Truncus Arteriosus	27	0.39
11.Persistant fetal circulation (PFC)	16	0.23
12.Ebstein anomaly	10	0.14
13. Mitral Atresia	09	0.13
14.Aorto pulmonary window (AP Window)	8	0.12

CHD with associated syndromes

Name of syndrome	Number	%
1. Down syndrome	166	2.4
2. Congenital Rubella syndrome (CRS)	68	0.98
3. Noonan’s syndrome	20	0.29
4.Williams Beuren syndrome	12	0.17
5.Turners syndrome	5	0.07
6.Golder Har syndrome	4	0.06
7.Edward syndrome	2	0.29
8.other	4	0.06

Types of intervention in study cases

Name of intervention	Number	%
1.PDA device closure	296	4.28
2.ASD device closure	206	2.89
3.VSD device closure	28	0.40
4.Pulmunar Balloon valvoplasty	23	0.34
5. Balloon atrial septostomy	11	0.16
6. Arotic Balloon valvoplasty	9	0.13
7.Balloon Coarctoplasty	8	0.12
8.PDA stenting	8	0.12
9. RVOT stenting and stenting from VSD to pulmonary artery	07 (5+2)	0.11
10. COA stenting	02	0.02
11. Pulmonary valve Implantation with Melody	01	0.01
Total intervention	599	8.66

Surgical treatment of study cases

Name of surgery	Number	Percentage
1.VSD closure	173	2.5
2.ASD closure	107	1.5
3.TOF repair	74	1.07
4.Bidirectional Glenn shunt for SV physiology/anatomy	58	0.83
5. AV canal repair	35	0.50
6.Rerouting of TAPVC	22	0.32
7.Blalock Taussig shunt	21	0.31
8.Coarctation repair	19	0.27
9.Arterial switch operation for TGA (ASO)	14	0.20

10.Mital valve repair	6	0.87
11.Truncus repair	4	0.05
12. A-P window repair	3	0.04
13.PDA ligation	3	0.04
14. Tricuspid valve repair for Ebstein Anomaly	3	0.04
15.Pulmonary artery banding for (DILV )	3	0.04
16.Rastelli operation	2	0.03
17.Arotic valve repair	1	0.01
Total surgery	548	7.93

Distribution of patient referred to cardiac surgeons

Outcome of VSD cases (n=1286)

Outcome of ASD & PFO (n=1066+265=1331)

Outcome of PDA (n=594)

Follow up of cases in 1st year

Follow up	No	Percentage
Reported	6444	93.20
Not reported	470	6.80
Spontaneous cure/ cured with medical treatment	678	9.80

SUMMARY OF OBSERVATION

- Most of the patient in this study were in >1 month to 1 year age group (36.62%)
- Median age of severe CHD was 11 years
- Male outnumbered female (55.73%)
- CHD prevalence is high in female adults.
- Disease pattern in study groups showed-
  - VSD is the commonest type of CHD (18.60%)
  - Followed by ASD (15.42%)
  - Then PDA (8.59%)

Among the cyanotic heart disease TOF is the commonest of all.
Among genetic & chromosomal defect Down syndrome was the commonest in our series (2.4%)

Patterns of management

Interventional technique (n=599/ 8.66%)

Commonest – PDA device closure (n=296/4.28%)

2nd highest – ASD device closure (2.89%)

Surgical procedure (n=548/ 7.93%)

Commonest – VSD closure

Then – ASD closure, TOF repair, BDG shunt, AV canal repair

Pattern of individual congenital heart disease in Bangladeshi children

VSD (1286 ,18.60%)

Spontaneous closure occurs in 23.33% cases by 3 year of age, Large PM & muscular VSD were not closed spontaneously and Common complications are –

Eisenmenger syndrome (3%)

Infundibular stenosis (3%)

AR(5%)

Small VSD with big shunt (1%)

ASD (1066 , 15.41%)

Incidence of ASD is influenced by timing of ECHO as
- Many ASD <5 mm closed spontaneously
- Many child escaped early diagnosis due to lack of symptoms and physical sign
- Moreover many patient with PFO are included in ASD which increases the incidence.
ASD device closure success rate is high in children than adults.

PDA(594, 8.59%)

Special features in Bangladeshi children are –
- Most PDA are closed by 7 days (term & preterm)
- Large tubular PDA is common in Down syndrome, Rubella syndrome
- Tubular PDA had rapid Eisenmenger change
These tubular PDAs are closed with device in Bangladesh in Armed Forces Center and LABAID cardiac center.

TOF ( 331,4.79%)

Surgical correction has done mostly in neighboring countries.
Developed pulmonary regurgitation in most cases following surgery
Valve replacement, redo surgery & surgery with RV to PA conduit was performed in some study cases.
Conduit change was required in 6 cases
Pulmonary Valve replacement with Melody valve was done in 1 cases.

TGA(156,2.26%)

TGA with ASD II0 & PDA is common than TGA with VSD in Bangladeshi Children.
Taussig Bing Anomaly was seen in some cases of double outlet right ventricle which resembles TGA.
Newborn with TGA do very well after septostomy.

Pulmonary Atresia(108,1.56%

Frequency of PA is 108 (1.56%), most of them had associated VSD.
PA with intact ventricular septum was less common
Major Aorto-pulmonary collaterals (MAPCA) were a common association with VSD
PDA was vertical and tortuous in most cases of PA with VSD cases and was difficult for stenting
In PA with intact IVS, PDA was classical & stenting was successful in all of these cases
BAS was done in few cases.

Single ventricle of LV type(49, 0 .71%)

Those with restrictive pulmonary blood flow usually remain stable for long time
Those with TGA or non restrictive pulmonary flow developed heart failure
Double inlet left ventricle (0.44%) was common of these group in our study.

TA (62, 0.89%)

Some cases are associated with
- TGA
- PS
- PA
All cases were planned for BDG shunt
If increased pulmonary artery flow – PA banding was advised
Two cases with restrictive VSD had stenting from VSD to pulmonary artery.

Common mistakes in ECHO in Bangladeshi centers

COA
Persistent pulmonary hypertension of newborn
Persistent fetal circulation
Large VSD with reverse shunt
Comments on ventriculo arterial relationship in complex cases
Differentiation of truncus arteriosus and pulmonary atresia
Anomalous origin of left coronary artery from pulmonary artery (ALCAPA)
Coronary artery fistula
Atretic condition of valves
Aortico left ventricular tunnel (ALVT)
Ruptured sinus of valsalva

Special points

DORV , VSD with PS

Round face, round eye, prominent forehead

Congenital rubella syndrome

New invention

Protocol formulated by us for Persistent Pulmonary Hypertension in 2001 has success rate > 99%
Large & huge tubular PDA is closed by device closure in Armed Forces Pediatric cardiac center since 2008.
Perimembranous VSD was closed by ADO II device from retrograde approach is practiced in 100% cases of small VSD’s. This method was innovated by pediatric cardiac team of Armed Forces Pediatric Cardiac Center spontaneously while doing an adult female case in 2012.