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Large bilateral adrenal haemorrhages in a newborn with unrepaired cyanotic CHD

Published online by Cambridge University Press:  08 April 2016

Lerraughn M. Morgan Open the ORCID record for Lerraughn M. Morgan [Opens in a new window] ,
Erle H. Austin III  and
Brian J. Holland
Lerraughn M. Morgan*
Affiliation:
Department of Pediatrics, Office of Medical Education, Kosair Children’s Hospital, University of Louisville, Louisville, Kentucky, United States of America
Erle H. Austin III
Affiliation:
Department of Cardiovascular and Thoracic Surgery, University of Louisville, Louisville, Kentucky, United States of America
Brian J. Holland
Affiliation:
Division of Pediatric Cardiology, University of Louisville, Louisville, Kentucky, United States of America
*
Correspondence to: L. M. Morgan, DO, Department of Pediatrics, Office of Medical Education, Kosair Children’s Hospital, University of Louisville, 231 East Chestnut Street, K609 (6th floor), Louisville, KY 40202, United States of America. Tel: +502 629 8828; Fax: +502 629 6783; E-mail: lmmorg05@louisville.edu
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Abstract

Management of newborns with cyanotic CHD and bilateral adrenal haemorrhages has not previously been described in the literature. These abnormalities present unique challenges due to the potential for haemodynamic instability, need for open heart surgery and associated systemic anticoagulation in the newborn period, and the risk of catastrophic bleeding.

Keywords

Transposition of the great arteriesCHDadrenal haemorrhageanticoagulation

Information

Type
Brief Reports
Information
Cardiology in the Young , Volume 26 , Issue 5 , June 2016 , pp. 1005 - 1007
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Copyright
© Cambridge University Press 2016 

CHD are the most common birth defects, with a prevalence of approximately eight per 1000 live births. Critical CHD, which requires newborn intervention and includes d-transposition of the great arteries, has an incidence of 2.5–3/1000 live births.Reference Hoffman and Kaplan 1 , Reference van der Linde, Konings and Slager 2 The prevalence of d-transposition of the great arteries is ~2.4/10,000 births.Reference Silerbach and Hannon 3 Once this diagnosis is confirmed, an open heart neonatal repair called the arterial switch operation is the standard of care.Reference Stoica, Carpenter and Campbell 4

Case

A male infant born at 40 6/7 weeks of gestation via vaginal delivery at a community hospital developed cyanosis with an oxygen saturation of 79%, despite 2 L of supplemental oxygen. There were no prenatal risk factors, the pregnancy was uncomplicated, and Apgar scores were 8 and 8 at 1 and 5 minutes, respectively. He was transferred to our facility, and on arrival he was cyanotic; his cardiac examination was notable for normal S1 and S2, no murmur, and distal perfusion was normal. His capillary blood gas was pH 7.28, pCO2 48, pO2 28, and HCO3 22. His white blood cell count was 29.1 K/μl with 40% neutrophils, haemoglobin 20.8 g/dl, haematocrit 61%, and platelets 157,000 K/μl. Empiric antibiotics were initiated, and a chest X-ray showed cardiomegaly. Prostaglandin E1 infusion was initiated because of the clinical impression of cyanotic CHD.

An echocardiogram demonstrated d-transposition of the great arteries with an intact ventricular septum (Fig 1). An echocardiography-guided balloon atrial septostomy was performed at the bedside, resulting in increased oxygen saturations up to 95%. Following the procedure, the patient was hypotensive with systolic blood pressure of 47 mmHg, leading to initiation of dopamine infusion. Owing to persistent hypotension without a clear aetiology, a renal ultrasound was obtained on day of life 2, which demonstrated large cystic structures in the bilateral adrenal regions. Further evaluation yielded normal prothrombin time, partial thromboplastin time, and fibrinogen. Urine catecholamines were within normal limits, but cortisol level was low (14.1 μg/dl). On the basis of imaging studies and clinical findings, a diagnosis of large bilateral adrenal haemorrhages with adrenal insufficiency was made. On day of life 10, an MRI of the abdomen confirmed this diagnosis and showed cystic bilateral adrenal masses measuring 3.8 cm in diameter on the right and 4.3 cm on the left (Fig 2).

Figure 1

Echocardiogram demonstrating d-transposition of the great arteries. AO=aorta; LV=left ventricle; PA=pulmonary artery; RV=right ventricle.

Figure 2

Non-contrast, multiplanar, and multisequence MRI of the abdomen demonstrating bilateral subacute adrenal haemorrhages. LAH=left adrenal haemorrhage; RAH=right adrenal haemorrhage.

Serial renal ultrasounds showed increasing size of the adrenal haemorrhages for the first 10 days. On the basis of guidelines for restarting systemic anticoagulation following stroke and intracerebral haemorrhage in adults,Reference Goldstein and Greenberg 5 and consensus among providers in our ICU, the decision was made to perform cardiac surgery once the adrenal haemorrhages had stabilised for 7 days. The patient was started on corticosteroid replacement with fludrocortisone. His blood pressure stabilised and intravenous pressors were eventually discontinued. Furosemide was started for pulmonary over-circulation. The patient received a stress dose of hydrocortisone, and then underwent an arterial switch operation with patent ductus arteriosus ligation and division with primary closure of the atrial septal defect on day of life 16 using cardiopulmonary bypass without complications. The post-operative course was complicated by feeding difficulties requiring placement of a gastrostomy tube, respiratory insufficiency, chylothorax, and left hemidiaphragm paralysis. Post-operative serial renal ultrasounds demonstrated decreasing size of both adrenal haemorrhages. The patient was discharged home on day of life 74 in good condition taking full enteral feedings and fludrocortisone.

Discussion

To the best of our knowledge, this is the first reported case of bilateral adrenal haemorrhage in a newborn with unrepaired critical CHD.

Adrenal haemorrhage, the most common adrenal mass in newborns, typically occurs within the first few days of life, when the size of the adrenals is relatively large. Aetiologies include traumatic events, heparin-induced thrombocytopaenia, maternal diabetes, or hypoxic events in utero.Reference Navarro and Daneman 6 , Reference Velaphi and Perlman 7 The aetiology of our patient’s adrenal haemorrhages is unknown, as the history was not significant for fetal distress or hypoxia in utero. Adrenal haemorrhage has the potential to affect adrenal gland function and contribute to adrenal insufficiency.Reference Oelkers 8 Identifying and addressing adrenal haemorrhage with steroid replacement avoided full adrenal and vascular collapse.

The prolonged pre-operative course raised concerns regarding the risk of inadequate systemic perfusion and oxygenation before surgery, as well as the risk of increased post-operative morbidity. The arterial switch operation is usually conducted early in the newborn period, with mean age at time of operation of 4–7 days and wait times before surgery of 2–4 days.Reference Kumar, Newburger and Gauvreau 9 Recently, Anderson et alReference Anderson, Ciarleglio and Hayes 10 looked at outcomes for neonates with d-transposition of the great arteries and found increased morbidity in neonates who had the operation after 3 days of age. In this case, cardiac surgical repair was postponed until the patient’s adrenal haemorrhages had stabilised, in order to minimise the potential risk of increased adrenal haemorrhages caused by systemic anticoagulation required during cardiopulmonary bypass. Our decision to postpone cardiac surgery for a week after stabilisation of the adrenal haemorrhages was based on previous research in adults, which looked at the risk of worsened bleeding when starting systemic anticoagulation after intracerebral haemorrhages. The American Heart Association suggests restarting anticoagulation 7–10 days after intracerebral haemorrhage stabilises.Reference Goldstein and Greenberg 5 Our patient had an uneventful intraoperative and post-operative course from a cardiovascular standpoint, had gradual decrease in size of both adrenal haemorrhages, and did well at outpatient follow-up.

Conclusion

We report a unique case of a term newborn with d-transposition of the great arteries and bilateral adrenal haemorrhages. In this case, we were able to successfully provide medical management for d-transposition of the great arteries and adrenal insufficiency beyond the usual surgical window of 3–7 days of age. We also safely provided systemic anticoagulation once bleeding had stabilised for 7 days. This approach may be considered for other children with haemorrhages who require systemic anticoagulation.

Acknowledgements

The authors thank Ms Elizabeth Smigielski for literature review assistance.

Financial Support

This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.

Conflicts of Interest

None.

References

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Figure 0

Figure 1 Echocardiogram demonstrating d-transposition of the great arteries. AO=aorta; LV=left ventricle; PA=pulmonary artery; RV=right ventricle.

Figure 1

Figure 2 Non-contrast, multiplanar, and multisequence MRI of the abdomen demonstrating bilateral subacute adrenal haemorrhages. LAH=left adrenal haemorrhage; RAH=right adrenal haemorrhage.