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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 6  |  Issue : 2  |  Page : 123-125

Multiple structural cardiac malformations in a macrosomic, possibly missed infant of a diabetic mother


1 The Limi Children's Hospital/CardioCare Cardiovascular Specialty Hospital, Abuja-FCT, Kano, Nigeria
2 Department of Paediatrics, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria

Date of Submission12-Apr-2018
Date of Decision16-May-2018
Date of Acceptance27-May-2018
Date of Web Publication23-May-2019

Correspondence Address:
Igoche David Peter
The Limi Children's Hospital/CardioCare Cardiovascular Specialty Hospital, Abuja-FCT
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/cjhr.cjhr_47_18

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  Abstract 


Infants of diabetic mothers are prone to cardiac malformations due to teratogenicity of diabetes mellitus (DM). We present here the case of a macrosomic neonate who had marked difficulty with breathing. He had severe hypertrophic cardiomyopathy with patent ductus arteriosus and cor triatriatum dexter, and although his mother had obstetric and a family history suggestive of DM, this diagnosis was not made as her antenatal fasting plasma glucose assay was normal. The need for adequate diagnosis using oral glucose tolerance test or hemoglobin A1c is hereby emphasized.

Keywords: Cor triatriatrium dextrum, hypertrophic cardiomyopathy, nondiabetic mother, patent ductus arteriosus


How to cite this article:
Peter ID, Farouk ZL, Mohammed A, Asani MO, Aliyu I, Usman F. Multiple structural cardiac malformations in a macrosomic, possibly missed infant of a diabetic mother. CHRISMED J Health Res 2019;6:123-5

How to cite this URL:
Peter ID, Farouk ZL, Mohammed A, Asani MO, Aliyu I, Usman F. Multiple structural cardiac malformations in a macrosomic, possibly missed infant of a diabetic mother. CHRISMED J Health Res [serial online] 2019 [cited 2019 Oct 17];6:123-5. Available from: http://www.cjhr.org/text.asp?2019/6/2/123/258979




  Introduction Top


Macrosomia, defined as a neonate's weight in excess of 4,000 g at birth, is the classic presentation of the infant of a diabetic mother (IDM).[1] It results from the biochemical events along the maternal hyperglycemia–fetal hyperinsulinemia pathway, although a few IDMs may be small for gestational age.[2] IDMs are more likely to have congenital anomalies when compared with infants of nondiabetic mothers.[1] The risk of congenital malformations is as high as 12% in IDMs, with congenital heart defects being the most common of these.[3],[4] Of these cardiac defects in IDMs, hypertrophic cardiomyopathy (HCM) has been identified as the most common, although existing literature on severe HCM (defined as having an end-diastolic interventricular septal thickness (IVSd) Z-score >2) in IDMs is scanty, especially when gestational diabetes mellitus (GDM) is unconfirmed.[5],[6] Existing literature has reported only five patients with severe neonatal HCM in association with GDM;[7],[8],[9] however, no such case has been reported among Nigerians to the best of the authors' knowledge. Although Otaigbe and Tabansi[10] reported HCM in an infant of a nondiabetic mother, this case was not severe and they did not associate this with maternal glycemic status.

It has been observed in IDMs that there is a delay in the closure of ductus arteriosus and decrease in pulmonary artery pressure after birth in comparison with controls.[11] This likely accounts for the high incidence of patent ductus arteriosus (PDA) neonates whose mothers are suffering from DM.[12]

We are reporting a macrosomic neonate with multiple structural cardiac lesions (severe HCM, PDA, and cor triatriatum dextrum) whose mother (with a suspicious obstetric history), probably had an undiagnosed GDM.


  Case Report Top


A 2 hour old neonate presented at the special care baby unit of the Aminu Kano Teaching Hospital, Kano, with complaints of marked difficulty with breathing. He was delivered by spontaneous vertex delivery at home and cried well at birth. Antenatal care was at a secondary health facility, and this period was not adversely eventful. His 26-year-old mother who had three previous macrosomic babies with one stillbirth and a positive family history of diabetes in maternal grandfather reported normal routine antenatal investigation results including fasting plasma glucose levels. She did not know the result of the fetal ultrasound scan done and had no toxic exposure in pregnancy. The baby was macrosomic (weight 4,600 g) with no dysmorphic features. He was cyanosed and dyspneic at birth. Apgar score was not available as the baby was delivered at home with no medical supervision; however, he was said to have cried well at birth. Estimated gestational age at presentation was 40 weeks, with length of 55 cm and a body surface area (BSA) of 0.3 m2. He was tachypneic with a respiratory rate of 64/min with a pulse oximetry reading of 54% in room air and 92% on intranasal oxygen and a heart rate of 160/min. The apex beat was displaced to the fifth intercostal space at the anterior axillary line; the first, second, and third heart sounds with a continuous murmur at the upper left sternal margin were auscultated. There was tender hepatomegaly up to 4 cm below the right costal margin. Cardiac silhouette was enlarged with pulmonary venous congestion on chest radiograph. Transthoracic echocardiogram revealed a fenestrated membranous septum dividing the right atrium into two chambers [Figure 1]. There was hypertrophy of the free walls of both ventricles with asymmetrical septal hypertrophy and narrowing of both ventricular cavities on 2-D [Figure 2]. On M-mode, IVSd measured 1 cm while expected based on BSA of 0.3 m2 is 0.40 cm (Z-score >3), systolic anterior motion of the mitral valve was evident, and systolic function was hyperdynamic with ejection fraction of 90% [Figure 3]. Patency of the arterial ductus was evident with a rooftop pattern seen on continuous-wave Doppler echocardiography [Figure 4]. Echo conclusion was that of severe HCM with cor triatriatum dexter and PDA. Other investigation included a random plasma glucose of 1.8 mmol/L at admission which was corrected, and he remained euglycemic thereafter. The full blood count was suggestive of sepsis with leukocytosis of 35.2 × 109/L and lymphocyte predominance (18.0 × 109/L) and a granulocyte count of 13.8 × 109/L; hematocrit and platelet levels were essentially normal (44.4% and 187 × 109/L). There are no facilities for genetic testing for etiology of HCM at our center. He was managed for congestive heart failure in a macrosomic infant with severe HCM with PDA and cor triatriatum dexter with early-onset neonatal sepsis. He was nursed in cardiac position and received intranasal oxygen, expressed breast milk through nasogastric tube, propranolol and furosemide, and discharged in stable condition after 12 days of hospitalization. He is being followed up in cardiology clinic on propranolol and diuretics and has remained stable with IVSd regressing (measuring 0.50 cm) at 5 weeks of life.
Figure 1: Subcostal view showing cor triatriatum dexter

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Figure 2: Parasternal long-axis view showing hypertrophy of the free walls of both ventricles with asymmetrical septal hypertrophy and narrowing of both ventricular cavities on two-dimensional imaging

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Figure 3: M-mode echocardiography showing systolic anterior motion of the mitral valve and a hyperdynamic systolic function

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Figure 4: Parasternal short-axis view showing rooftop pattern of patent ductus arteriosus seen on continuous-wave Doppler echocardiography

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  Discussion Top


The mother of our patient was neither previously diagnosed with pre-GDM nor was GDM diagnosed during her antenatal period from screening using fasting plasma glucose assay despite the pointers to this possibility from the past medical and obstetric history. HCM in IDMs is known to resolve at 4 months of life, and the gradual resolution of HCM noted at 5th week of life further suggest that the baby could be an IDM. Suffice it to say that fasting plasma glucose is not a good screening test for GDM.[13] The oral glucose tolerance test or hemoglobin A1c (HbA1c) which has been adopted by the WHO as a diagnostic tool for DM may have unveiled the existence of this in the mother of this neonate.[14] We were limited by the absence of facilities for genetic screening in our center as Vincent et al.,[5] who reported three children with macrosomia and severe HCM, hypothesized that additional factors to maternal diabetes, such as genetic and/or metabolic factors, might be involved in the determination of this phenotype. In the cases reported by them, however, history and laboratory pointers to maternal DM were negative.

Abu-Sulaiman and Subaih[12] found that PDA was the most common heart lesion, occurring in 70% of 100 Saudi IDMs studied, and HCM was observed in 38% of these, meaning some may have had an overlap of the two as our patient had. Our patient who may have been an unidentified IDM also had three structural cardiac malformations. The presence of cor triatriatum dexter in our patient may have accounted for the cyanosis observed as was reported by Asani et al.[15]


  Conclusion Top


A unique case of a macrosomic neonate with multiple structural cardiac malformations (including severe HCM with PDA and cor triatriatum dexter) who may have been a missed IDM has been reported. It is, therefore, necessary that physicians are more enlightened on recommended diagnostic tests for GDM using oral glucose tolerance test or HbA1c and not just fasting plasma glucose assay.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Lee-Parritz A, Cloherty JP. Diabetes mellitus. In: Cloherty JP, Eichenwald EC, Stark AR, editors. Manual of Neonatal Care. 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2008. p. 10-9.  Back to cited text no. 1
    
2.
Heritage CK. Infant of a diabetic mother. In: Neonatology: Management, Procedures, On-Call Problems, Diseases, and Drugs. 5th ed. USA: The McGraw-Hill Companies; 2004. p. 418-23.  Back to cited text no. 2
    
3.
Narchi H, Kulaylat N. Heart disease in infants of diabetic mothers. Images Paediatr Cardiol 2000;2:17-23.  Back to cited text no. 3
    
4.
Ramos-Arroyo MA, Rodriguez-Pinilla E, Cordero JF. Maternal diabetes: The risk for specific birth defects. Eur J Epidemiol 1992;8:503-8.  Back to cited text no. 4
    
5.
Vincent M, Benbrik N, Romefort B, Colombel A, Bézieau S, Isidor B, et al. Three patients presenting with severe macrosomia and congenital hypertrophic cardiomyopathy: A case series. J Med Case Rep 2017;11:78.  Back to cited text no. 5
    
6.
Maron BJ, Clark CE, Epstein SE. Asymmetric septal hypertrophy: Echocardiographic identification of the pathognomonic anatomic abnormality of IHSS. Circulation 1977;47:233.  Back to cited text no. 6
    
7.
Franzese A, Valerio G, Ciccarelli NP, De Filippo G, Iannucci MP, Alfonsi L, et al. Severe hypertrophic cardiomyopathy in an infant of a diabetic mother. Diabetes Care 1997;20:676-7.  Back to cited text no. 7
    
8.
McMahon JN, Berry PJ, Joffe HS. Fatal hypertrophic cardiomyopathy in an infant of a diabetic mother. Pediatr Cardiol 1990;11:211-2.  Back to cited text no. 8
    
9.
Robinson B, Eshaghpour E, Ewing S, Baumgart S. Hypertrophic obstructive cardiomyopathy in an infant of a diabetic mother: Support by extracorporeal membrane oxygenation and treatment with beta-adrenergic blockade and increased intravenous fluid administration. ASAIO J 1998;44:845-7.  Back to cited text no. 9
    
10.
Otaigbe BE, Tabansi PN. Congenital heart disease in the Niger delta region of Nigeria: A four-year prospective echocardiographic analysis. Cardiovasc J Afr 2014;25:265-8.  Back to cited text no. 10
    
11.
Vela-Huerta M, Aguilera-López A, Alarcón-Santos S, Amador N, Aldana-Valenzuela C, Heredia A, et al. Cardiopulmonary adaptation in large for gestational age infants of diabetic and nondiabetic mothers. Acta Paediatr 2007;96:1303-7.  Back to cited text no. 11
    
12.
Abu-Sulaiman RM, Subaih B. Congenital heart disease in infants of diabetic mothers: Echocardiographic study. Pediatr Cardiol 2004;25:137-40.  Back to cited text no. 12
    
13.
Taylor R, Zimmet P. Limitation of fasting plasma glucose for the diagnosis of diabetes mellitus. Diabetes Care 1981;4:556-8.  Back to cited text no. 13
    
14.
Rajput R, Yadav Y, Rajput M, Nanda S. Utility of hbA1c for diagnosis of gestational diabetes mellitus. Diabetes Res Clin Pract 2012;98:104-7.  Back to cited text no. 14
    
15.
Asani OM, Gambo S, Peter I. Cor triatriatum dexter with pulmonary hypertension. Sahel Med J 2016;19:44-6.  Back to cited text no. 15
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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