|Year : 2016 | Volume
| Issue : 2 | Page : 257-260
Asymmetrical septal hypertrophy and hypertrophic cardiomyopathy in infant of diabetic mother: A reversible cardiomyopathy
Deepak Sharma1, Aakash Pandita2, Sweta Shastri3, Pradeep Sharma4
1 Department of Paediatrics, PGIMS, Rohtak, Haryana
2 Department of Paediatrics, Government Medical College, Jammu, Jammu and Kashmir
3 Department of Pathology, N.K.P Salve Medical College, Nagpur, Maharasthra
4 Department of Pathology, RNT Medical College, Udaipur, Rajasthan
|Date of Web Publication||1-Mar-2016|
Department of Paediatrics, PGIMS, Rohtak, Haryana
Source of Support: None, Conflict of Interest: None
Hypertrophic cardiomyopathy (HOCM) that includes thickening of one or both of the ventricular walls, hypertrophy of the interventricular septum; systolic and diastolic dysfunction, transient hypertrophic sub-aortic stenosis is a well-recognized comorbidity in infants of diabetic mothers. This cardiac complication in the infant of the diabetic mother has recently been named as pathological ventricular hypertrophy to avoid confusion with the autosomal dominant form of congenital HCM. We report a neonate that was diagnosed as case of asymmetrical septal hypertrophy and hypertrophic cardiomyopathy and was born to mother who had poor glycemic control and was treated successfully with propranolol and showed resolution of hypertrophy in follow-up echocardiography.
Keywords: Asymmetrical septal hypertrophy, hypertrophic cardiomyopathy, infant of diabetic mother, propranolol
|How to cite this article:|
Sharma D, Pandita A, Shastri S, Sharma P. Asymmetrical septal hypertrophy and hypertrophic cardiomyopathy in infant of diabetic mother: A reversible cardiomyopathy. Med J DY Patil Univ 2016;9:257-60
|How to cite this URL:|
Sharma D, Pandita A, Shastri S, Sharma P. Asymmetrical septal hypertrophy and hypertrophic cardiomyopathy in infant of diabetic mother: A reversible cardiomyopathy. Med J DY Patil Univ [serial online] 2016 [cited 2020 Sep 18];9:257-60. Available from: http://www.mjdrdypu.org/text.asp?2016/9/2/257/177679
| Introduction|| |
Gestational diabetes mellitus (GDM) from all causes of diabetes is the most common medical complication seen during pregnancy and the incidence is in increase whole over the world including developed and developing countries especially this is seen in incidence of type 2 diabetes mellitus. Despite the recent advances in perinatal care around the globe, infants of diabetic mothers (IDMs) still remain at risk of multiple of problems in the immediate neonatal period that includes various physiologic, metabolic, and congenital complications namely preterm birth, birth asphyxia, macrosomia, respiratory distress including hyaline membrane disease and transient tachypnea of newborn (TTNB), hypoglycemia, hypocalcemia, hyperbilirubinemia, polycythemia and hyperviscosity, hypertrophic cardiomyopathy, asymmetrical septal hypertrophy and congenital anomalies, particularly of the central nervous system. , The type 1 diabetes around the time of conception produces marked risk of embryopathy (neural tube defects, cardiac defects, and caudal regression syndrome) as these mother have high glycosylated hemoglobin at the time of embryogenesis (around 6-8 weeks of gestation) rather than IDM that are born to type 2 diabetes mothers who have macrosomia and other milder problems. The mothers who have been diagnosed as severe and unstable type 1 diabetes in the later part of gestation have high chances of neonate being more effected with intrauterine growth restriction, asphyxia, and fetal death. ,, We report a neonate that was diagnosed as case of asymmetrical septal hypertrophy and hypertrophic cardiomyopathy and was born to mother who had poor glycemic control and was treated successfully with propranolol and showed resolution of hypertrophy in follow-up echocardiography.
| Case Report|| |
A term male baby (38 weeks) with a birth weight of 4.5 kg with normal Apgar scores of 8/9/9 at 1, 5 and 10 min respectively was born to 34-year-old G2P2 L1 mother by caesarean section. The mother was a known case of GDM with sugar being controlled on Insulin. The glycosylated hemoglobin done in second trimester showed the value of 9.4%. The infant was admitted in the nursery in view of the respiratory distress and for sugar monitoring. The physical examination finding showed a macrosomic infant (weight >95% centile for age), hairy pinna, thick upper extremity skin folds, broad shoulder and torso with a relatively smaller head, and plethoric look. There were no other obvious malformations. The provisional diagnosis of TTNB was kept, and the infant was started on hood oxygen. Chest radiograph was suggestive of features related to TTNB and with cardiomegaly (cardio-thoracic ratio of 70%). The baby was evaluated with serum calcium, sugar and packed cell volume, which showed normal values. The Baby was evaluated with 2D echocardiography to rule out associated cardiac anomalies. The echocardiography done was suggestive of biventricular hypertrophy with marked interventricular septal hypertrophy with a significant reduction in the ventricular cavity, with features suggestive of hypertrophic cardiomyopathy (HOCM) [Figure 1] and [Figure 2]. There were no associated cardiac malformations. The infant was started on oral propranolol at the dose of 1 mg/kg/dose every 8 th hourly with regular monitoring of sugar and heart rate. There was a gradual improvement in respiratory distress in over next 24 h. Hence baby was started on breastfeeding by next day. The infant was discharged in well condition and was in the regular follow-up. The repeat echo done at the age of 4 months showed resolution of hypertrophy [Figure 3] hence propranolol was stopped.
|Figures 1: Figure showing echocardiographic findings of biventricular hypertrophy with marked interventricular septal hypertrophy with signifi cant reduction in volume of ventricular cavity, features suggestive of hypertrophic cardiomyopathy|
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|Figure 2: Figure showing echocardiographic findings of biventricular hypertrophy with marked interventricular septal hypertrophy with significant reduction in volume of ventricular cavity, features suggestive of hypertrophic cardiomyopathy|
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|Figure 3: Figure showing echocardiographic fi ndings of resolution of the hypertrophy at the age of 4 months|
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| Discussion|| |
Diabetes in pregnancy usually results in an increased risk of fetal, neonatal, and long term complications in the newborn with most important complication being diabetic embryopathy resulting in numerous congenital anomalies. Maternal diabetes is of many types like pregestational (i.e., type 1 or type 2 diabetes which is being diagnosed before conception) or gestational (i.e., diabetes diagnosed during pregnancy).  The fetal outcome is usually related to the age of onset and duration of glucose intolerance during pregnancy and severity of the mother's diabetes. The various fetal complications seen are listed in [Table 1]. ,,
The various neonatal complications in offspring of diabetic mothers are enlisted in [Table 2]. ,,]
The diabetic cardiomyopathy is usually characterized with significant thickening of the interventricular septum leading to reduction in the size of the ventricular chambers, causing transient hypertrophic sub aortic stenosis.  There can be associated biventricular hypertrophy also and sometimes rarely right ventricular hypertrophy.  Aortic outflow obstruction is aggravated by anterior systolic motion of the mitral valve. Usually the infants are asymptomatic, but 5-10% have respiratory distress or signs of poor cardiac output or heart failure. The chest radiograph shows cardiomegaly, as in our case had and cardiac hypertrophy is best detected by 2D echocardiography. The echo shows hypertrophy of ventricular septum, the right anterior wall and left ventricular posterior wall. The diabetic cardiomyopathy is transient and this property makes it unique over other cardiomyopathy. It usually resolves with normalization of plasma insulin levels. The affected infants usually recover within 2 to 3 weeks of supportive care, and echocardiographic findings shows normalization within 6-12 months. Supportive care includes increased intravenous fluid administration and beta blockers like propranolol. Inotropic agents like dopamine and dobutamine are contraindicated as they are prone to decrease ventricular size and further obstruct cardiac outflow.  The pathophysiology of cardiac hypertrophy in infant of diabetic mother is explained by Pederson maternal hyperglycemia-fetal hyperinsulinemia hypothesis. The fetal hyperinsulinemia, leads to increase in the synthesis and deposition of fat and glycogen in the myocardial cells.  It is usually seen in mothers with poor glycemic control during pregnancy. ,
| Conclusion|| |
Infant of diabetic mothers are prone to have multiple problems during the neonatal period which makes them a very high risk infant. These neonates must be screened for any associated congenital anomalies and metabolic abnormalities. Hypertrophic cardiomyopathy of IDM have good prognosis and this cardiomyopathy is reversible in nature but these neonates requires close monitoring and regular follow-up. Beta blockers like propranolol can be started if the HOCM is symptomatic I the neonate but these must be monitored for side-effects like hypoglycemia and bradycardia.
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[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2]