Nigerian Journal of Paediatrics

Echocardiographic findings in newborns and postneonatal infants undergoing reoperative evaluation for surgically correctable non cardiac congenital malformations

Niger J Paediatr 2018; 45 (4):192 – 195

ORIGINAL

Onalo R

Osagie OO

CC – BY Echocardiographic findings in

newborns and postneonatal

infants undergoing preoperative

evaluation for surgically

correctable non-cardiac congenital

malformations

DOI:http://dx.doi.org/10.4314/njp.v45i4.5

Accepted: 3rd May 2019

Abstract : Background: Congeni-

major and biliary atresias were the

tal cardiac malformations could

most common malformations seen.

Onalo R

(

)

co-exist with surgically correct-

Seven babies had completely nor-

Department of Paediatrics,

able non-cardiac congenital struc-

mal heart, five had isolated patent

University of Abuja Teaching

tural abnormalities. The occur-

foramen ovale, thus giving a total

Hospital, Gwagwalada, Abuja

rence of the two conditions por-

of 12 babies with structurally nor-

Nigeria.

tends increased anaesthetic risk

mal hearts. Of the 32 babies with

Email: richardonalo@yahoo.com

and perioperative complications.

structural heart defects, 17(53.1%)

Early recognition could favoura-

had single defect while 15 (46.9%)

Osagie OO

bly alter the post-operative out-

had two or more defects consisting

Department of Surgery,

come.

of 26 acyanotic and 4 cyanotic

University of Abuja Teaching

Objectives: To describe the preva-

defects. A total of 47 cardiac de-

Hospital,

lence and pattern of congenital

fects were seen. Forty-two (89.4%)

Gwagwalada, Abuja Nigeria.

heart defects in young infants

of the heart defects were simple

with extracardiac structural mal-

while 5(10.6%) were complex.

formations.

Atrial septal defect 13,(27.7%),

Methods: Consecutive newborns

patent

ductus

arteriosus

14,

and postneonatal infants with con-

(29.8%) and ventricular septal

genital malformations being pre-

defect 7,(14.9%) were the com-

pared for surgery were sent to the

monest simple heart defects while

paediatric cardiology unit of Uni-

atrioventricular septal defects pre-

versity of Abuja Teaching Hospi-

dominate among the complex cate-

tal for echocardiography and were

gory

recruited over a 30moth period. A

Conclusions: Congenital structural

transthoracic echocardiography,

abnormalities are common indica-

following the guidelines of the

tions for surgical interventions in

American Society of Echocardi-

young infants. Recognition of co-

ography, was used to evaluate the

existing structural cardiac abnor-

cardiac structures.

malities is essential in preoperative

Results: Of the 44 newborns and

planning and may be a useful

postneonatal infants with congeni-

guide in averting untoward anaes-

tal non-cardiac malformations, 27

thetic accident.

(61.4%) were males while 17

(38.6%) were females, giving a

Key words: Newborns, infants,

M:F ratio of 1.59:1. Gastrointesti-

congenital heart defects, extracar-

nal

malformations constituted

diac malformations

47.7% of cases. Omphalocoele

Introduction

tracheosophageal fistula, diaphragmatic hernia and in-

testinal atresias. Non-cardiac malformation was reported

Extracardiac structural malformations account for sig-

to be the immediate or major cause of death in 12% of

newborns in a study by Jaiyemisi and Antia. The contri-

nificant part of surgical consultations in the neonatal and

immediate postneonatal period mainly due to the gro-

bution of accompanying cardiac malformations to the

tesque appearance of those with the malformations espe-

mortality is however unknown.

cially omphalocoele major, myelomemningocoele and

bladder extrophy, as well as the unstable presentation of

Neonates and infants with congenital heart defects have

newborns with major internal malformations such as

a two-fold increase in mortality from non-cardiac sur-

193

gery. Anaesthesia-related paediatric cardiac arrest, ac-

Abnormalities of the digestive system constituted 47.7%

cording to a review, occurred in 75% of patients under 2

of the extracardiac malformations seen in the patients.

years of age with cardiac defects during non-cardiac

Omphalocoele major and intestinal atresias were the

surgery.

2,3

Nevertheless, in experienced hands, anaesthe-

most common of this malformation followed by biliary

sia for neonates with complex heart diseases presenting

atresia. Syndromic malformations and respiratory sys-

for treatment of general surgical emergencies can still be

tem anomaly contributed 18.2 and 11.4% respectively.

conducted with manageable complications.

Pattern of congenital malformations in the patients is

shown on Table 2.

Surgical amendment of the seemingly frightening exter-

nal congenital malformation has greatly altered the out-

Table 2: Pattern of extracardiac malformations among patients

come of management and the acceptance of the baby by

studied

parents. However, the management and prognosis of the

Type of extracardiac defects

Frequency

Percentages

patient may be determined by the presence or otherwise

Digestive System

47.7

of cardiac malformations especially at the perioperative

Omphalocoele

15.9

period where the cardiac defect may constitute a signifi-

Intestinal atresias + imperforate

15.9

cant anaesthetic risk and delay postoperative recovery.

anus

The present study aimed at describing the prevalence

Biliary atresia

9.0

and pattern of congenital heart defects in young infants

Inguinal hernia

2.3

with non-cardiac malformations preoperatively.

Cleft lip and palate

4.5

Syndromic Malformation

18.2

Down Syndrome

9.1

Pierre Robin Syndrome

4.5

Methodology

Edward Syndrome

2.3

Turner’s Syndrome

2.3

Consecutive newborns and postneonatal infants being

Respiratory System

11.4

prepared for surgery for non-cardiac malformations

Tracheosophageal fistula

2.9

were sent for echocardiographic evaluation and were

Diaphragmatic hernia

2.9

recruited for the study between March, 2015 and Sep-

Laryngomalacia

2.9

tember, 2017.

Genitourinary System

6.8

Bladder estrophy

4.5

Echocardiography was performed using a portable Gen-

Posterior urethral valve

2.3

eral Electric Vivid e echo machine fitted with a 6S (5-

Central nervous system

4.5

7MHz) transducer. Standard two-Dimensional, M-mode,

Thoracic myelomeningocoele

2.3

and Doppler echocardiograms were performed in the

Lumbosacral meningocoele

2.3

supine and left lateral decubitus positions and measure-

Skeletal System

9.1

ments done as recommended by the American Society

Polydactyly

2.3

of Echocardiography.

Multiple limb malformations

4.5

Ethical clearance was obtained for a study on structural

Achondroplasia

2.3

heart diseases in children and this study is part of a lar-

Vascular

2.3

ger one.

Giant haemangioma

2.3

Total

100

Results

The prevalence of cardiac defects among infants with

gastrointestinal anomaly was statistically significant,

( χ = 6.380, p = 0.029). However the association of car-

A total of 44 newborns and postneonatal infants with

non-cardiac malformations were referred for echocardi-

diac defects with other non-cardiac malformations failed

ography; 27 (61.4%) were males while 17 (38.6%) were

to reach statistical significance, Table 3.

females, giving a male-to-female ratio of 1.59:1. Eight-

Seven babies had completely normal heart, 5 had iso-

een (40.9%) of the patients presented within the first

lated patent foramen ovale, thus giving a total of 12 ba-

week of life. Details of the age at presentation are dis-

bies with structurally normal hearts. Of the 32(72.7%,

played in Table 1 which also shows the gender distribu-

32/44) babies with structural heart defects, 17 (53.1%)

tion of the patients studied.

had single defect while 15 (46.9%) had two or more

defects consisting of 26acyanotic and 4 cyanotic defects.

Table 1: Age and sex distribution of 44 young infants

A total of 47 cardiac defects were seen. Forty-two

with extra-cardiac malformations

(89.4%) of the heart defects were simple while 5

Age

Gender

Total

(10.6%) were complex heart defects. Atrial septal defect

Male (n = 27)

Female (n = 17)

N (%)

(13, 27.7%), patent ductus arteriosus (14, 29.8%) and

n (%)

ventricular septal defect (7, 14.9%) were the commonest

0-7days

11 (40.7)

7 (41.2)

18 (40.9)

simple heart defects while the comple category consists

8-14 days

4 (14.8)

3 (17.6)

7 (15.9)

of atrioventricular septal defect, double outlet right ven-

15-28 days

4 (14.8)

3 (17.6)

7 (15.9)

tricle, truncus arteriosus, mitral atresia with single ven-

tricle anatomy and total anomalous pulmonary venous

>28 days

8 (29.6)

4 (23.5)

12 (27.3)

return.

194

Distribution of cardiac defects is shown on Table 4 and

Table 3: Distribution of extra-cardiac anomaly among infants with congenital heart diseases

Extracardiac anomaly

Congenital heart disease

Total

Present

Absent

n=32

n=12

Digestive system

19 (59.4)

2 (16.7)

6.380

0.029

Identified syndrome

7 (21.9)

3 (25.0)

0.000

1.000

Respiratory system

2 (6.25)

3 (25.0)

1.469

0.225

Genitourinary system

2 (6.25)

1 (8.33)

FET

1.000

Central nervous system

2 (16.7)

FET

0.069

Others (Skeletal, haematologic)

2 (6.25)

1 (8.33)

FET

1.000

FET = Fishers exact test

Table 4: Echocardiographic diagnosis in 44 infants with extra-cardiac malformations

Echocardiographic diagno-

System involved in extracardiac malformations

sis

Digestive

G.U.S

Syndromic

Respiratory

CNS

Others δδ

Atrial Septal Defects

Ventricular Septal defects

Patent Ductus Arteriosus

Pulmonary Stenosis

Patent Foramen Ovale

Normal

ββ

Others

5**

** refers to Truncus arteriosus (1), Double outlet right ventricle (1), Total anomalous pulmo-

nary venous drainage (1), Mitral atresia (1), Aortic stenosis (1)

ββrefers to Atrioventricular septal defect (2), Left superior vena cava (1)

δδincluded giant haemangioma, skeletal abnormalities

G.U.S = Genitourinary system, CNS = Central nervous system

Table 5: Types of non-cardiac malfor mations seen in patients with complex cardiac defects

Non-cardiac malformation

Complex heart lesion

Down syndrome

Atrioventricular septal defect

Turner syndrome

Atrioventricular septal defect

Omphalocoele major

Double outlet right ventricle

Omphalocoele major

Mitral atresia with single ventricle anatomy

Diaphragmatic hernia

Truncus arteriosus

included children up to the age of 11years and it was

retrospective. In the series of Gonzalez et al from Cali-

Discussion

fornia, 50% of neonates with congenital heart defects

also had extracardiac anomaly, thus corroborating the

Anomaly of the digestive system constituted the major-

finding in the present study.

ity of abnormalities seen in the present study, similar to

the observation made by Oyati et al in Zaria but differs

The prevalence of cardiac anomaly in neonates with

from that of Jaiyesimi and Antia in Ibadan in which the

external non-cardiac malformations in this study sup-

prevalence of digestive system anomalies (23%) was

ports the need for preoperative echocardiography for this

superseded by musculoskeletal malformations (27%).

category of infants. Preoperative echocardiography

On the overall, anomaly of the digestive system such as

helps to identify the presence and type of cardiac defects

omphalocoele major were common in this subset of in-

as well as the severity of the malformations and hence

fants and tended to presents earlier, probably due to the

assists the surgeon, anaesthesiologist and neonatal cardi-

grotesque appearance of the baby.

5-7

ologist in weighing the risk-versus-benefit of the in-

tended surgery, vis-à-vis the ability of the patient to

The prevalence of cardiac defects among infants with

withstand the stress of surgery and even more critical,

extra-cardiac congenital malformations in the present

the stress of anaesthesia.

study is high (72.7%), twice the prevalence recorded in a

similar cohort in Zaria. The higher prevalence may be

Simple shunt defects were the most common type of

due to differences in study population. The Zaria study

cardiac defects in children with extra-cardiac malforma-

195

tions in this study. Atrial septal defects, ventricular sep-

The spectrum of cardiac defects documented in this

tal defects and patent ductus arteriosus predominated

study provides a guide in prognosticating the periopera-

among the simple heart lesions seen. Similar findings

tive outcome of newborns and postneonatal infants un-

have been reported elsewhere.

1,4-7

There is however no

dergoing surgery for non-cardiac malformations.

established association between type of extra-cardiac

anomaly and specific simple cardiac defect.

Complex cardiac defects were relatively rare, 10.6% of

Conclusions

all heart defects seen, and occurred in infants with syn-

dromic malformations and major gastrointestinal anom-

Cardiac malformations are common in young infants

aly. Although relatively rare, the presence of complex

presenting with extra-cardiac surgical disorders. All

cardiac defects in newborns with major gastrointestinal

newborns and immediate postneonatal infants undergo-

anomaly portends a precarious situation as the duo spells

ing corrective or palliative surgery for non-cardiac mal-

unfavourable outcome if not properly managed. How-

formations should have cardiac evaluation before com-

ever early identification of complex cardiac defects

mencement of the surgery.

helps in planning for the best treatment option within the

ambit of available resources.

Conflict of interest: None

Funding: None

References

1. Jaiyesimi F, Antia AU. Ex-

4. Oyati AI, Danbauchi SS, Ameh

6. Alabdulgader AA. Extracar-

tracardiac defects in children

EA, Mshelbwala PM, Anumah

diac anomalies associated with

with congenital heart disease.

MA, Ogunrinde GO, Anyiam

congenital cardiac malforma-

Br Heart J 1979; 42: 475-479 .

JO, Azuh PC. Echocardio-

tions in Saudi Arabian popula-

2. Menghraj SJ. Anaesthetic con-

graphic findings in children

tion. Res J Cardiol 2012; 5:

siderations in children with

with surgically correctable non-

12-19.

congenital heart disease under-

cardiac congenital anomalies.

7. Rosa RCM, Rossa RFM, Zen

going non-cardiac surgery.

Ann Trop Paediatr 2009; 29:

PRG, Paskulin GA. Congeni-

Indian J Anaesth 2012; 56: 491

41-44.

tal heart defects and extracar-

-495.

5. Gonzalez JH, Shirali GS, Atz

diac malformations. Rev Paul

3. Ramamoorthy C, Haberkern

AM, Taylor SN, Forbus GA,

Pediatr 2013; 31: 243-259.

CM, Bhananker SM, Domino

Zyblewski SC, et al. Universal

KB, Posner KL, Campos JS, et

screening for extracardiac ab-

al. Anaesthetic-related cardiac

normalities in neonates with

arrest in children with heart

congenital heart disease. Pedi-

disease: Data from the Pediat-

atr Cardiol 2009; 30: 269-273 .

ric Perioperative Cardiac Arrest

(POCA) registry. AnesthAnalg

2010; 110: 1376-1382.