Case - Infant of Diabetic Mother

Case by Dr Pasinee Kanaprach (fellow Neonatal Hemodynamics - McGill University) and Dr Juliana Quintero Salazar (fellow Neonatology - McGill University)

December 23, 2023

Case description

A 38-week pregnant woman with Type 1 diabetes and inadequately controlled insulin (HbA1c > 6.5) presented for an emergency C-section due to decreased fetal movements and abnormal fetal heart rate tracing. Routine ultrasounds were unremarkable, except for an estimated fetal weight (EFW) above the 97th percentile at the third trimester (T3) ultrasound (32+0 weeks). Fetal echocardiogram was normal.

The infant, born at 38 weeks via ventouse-assisted emergency C-section, exhibited respiratory distress, moderate encephalopathy, and hypoglycemia (lowest glucose 0.7). Resuscitation included positive pressure ventilation (PPV) for 2 minutes, chest compressions for 1 minute, and intubation. Apgar scores were 0-5-7-9. Cord pH was 7.08. The neonate was admitted to our NICU after medical transport.

Upon arrival, the neonate displayed signs of moderate encephalopathy, reduced spontaneous movements, and had a murmur. Chest X-ray revealed cardiomegaly. The neonate received D10W boluses and intravenous maintenance due to hypoglycemia. Therapeutic hypothermia was initiatied due to moderate encephalopathy. Inhaled nitrous oxide (iNO) was initiated for clinical pulmonary hypertension. An echocardiogram revealed severe biventricular hypertrophy, left ventricular outflow tract obstruction, and a large bidirectional patent ductus arteriosus (PDA). Propranolol was initiated at 2mg/kg/day after rewarming. Pulmonary hypertension resolved with subsequent echocardiograms. The neonate remained with normal blood pressure and had improving lactate levels. 

Therapeutic hypothermia was completed, and subsequent MRI on Day of Life (DOL) 10 revealed no signs of hypoxic-ischemic brain injury (no abnormalities on brain MRI). Follow-up echocardiography demonstrated progressive improvement in hypertrophy.

The planned dose of propranolol of 6mg/kg/day was not tolerated (transient bradycardia), and the final discharge dose was 4mg/kg/day, well tolerated with normal glycemia. 

Presentation on hypertrophic cardiomyopathy in the context of infant of diabetic mother

HOCM 2.pdf

Chest Radiographies

Chest radiography on admission: Confluent airspace opacities are seen. Increased central vascular lung markings. The cardio-thymic silhouette is enlarged. Abdomen unremarkable. The UVL is central. the UAL is curled.

Follow-up radiography: Lung volumes remain mildly enlarged. Findings of pulmonary edema persist. Improvement in the subsegmental atelectasis in the right. Cardiomegaly still present but improving.

Mechanisms of action of uncontrolled diabetes and anabolic effect

Reference: 

Manifestations of infant of diabetic mother

Echocardiography assessment

 Summary:

Severe biventricular hypertrophy with septal hypertrophy. Systolic anterior motion of the mitral valve, shrouding / obstructing the left ventricular outflow tract

Zoom on the LVOT - outlining that the aortic valve is opening and closing. This outlines that there is still some forward flow through the valve despite the obstructive picture. 

Intracavitary acceleration and acceleration through the LVOT during systole.

Biventricular hypertrophy observed in the short axis view. The pulmonary valve is seen anteriorly, opening and closing, outlining that there is flow through the right ventricular outflow tract despite the significant RV hypertrophy with narrowing of the RVOT during systole.

Narrowing of the LVOT relative to the ascending aorta.

Biventricular concentric hypertrophy.

Apical view showcasing the significant septal hypertrophy. There is near obliteration of the RV cavity in systole. 

Significant intracavitary acceleration (turbulence) in the right ventricle (Nyquiest at 1.19 - outlining the high velocities and aliasing). There is also some degree of mitral insufficiency, possibly secondary to the LV diastolic dysfunction due to the significant hypertrophy and likely decreased relaxation properties. 

Pulmonary veins are seen draining into the left atrium. 

The ductus arteriosus is seen and is bidirectional. In certain cases, it may be important to maintain the duct open. 

Significant hypertrophy observed in the apical view. 

Despite the significant hypertrophy, one may observe here the RVOT with opening and closing of the valve. The BP is 73/53 outlining that the baby is able to maintain appropriate output despite the diastolic dysfunction. 

Intracavitary acceleration in the LV with some flow going through the LVOT, despite the systolic anterior motion of the mitral valve. 

TAPSE is decreased outlining some degree of RV systolic dysfunction.

RV-RA outlining that the RV systolic pressure is isosystemic. 

The inter-atrial shunt is left to right and restrictive, with a velocity peak of 1.5  (gradient LA-RA of 9 mmHg).

TDI of LV septum indicating depressed s' and e', signs of systolic and diastolic dysfunction. 

TDI of LV free wall indicating depressed s' and e', signs of systolic and diastolic dysfunction. 

TDI of RV indicating depressed e', sign of systolic and diastolic dysfunction. 

SAM - Systolic anterior motion of the mitral valve. Significant hypertrophy outlined by the M-Mode at the tip of the mitral valve in PLAX.

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