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Cardiogenic Shock in a Toddler With Rickets
William A Harris1, John F Mobley2, Jessica E Hook1
1Division of Pediatric Cardiology, Medical University of South Carolina, Shawn Jenkins Children's Hospital, Charleston, South Carolina.
Insights
Severe vitamin D deficiency can cause dilated cardiomyopathy (DCM) in children. Prompt diagnosis and treatment, including mechanical support, can lead to recovery in these rare cases of pediatric DCM.
Area of Science:
- Pediatric Cardiology
- Pediatric Endocrinology
- Nutritional Deficiencies
Background:
- Dilated cardiomyopathy (DCM) in children is usually idiopathic, genetic, or infectious.
- Rarely, DCM can be secondary to vitamin D-deficient rickets, presenting with severe cardiac dysfunction.
- Severe rickets can manifest with hypocalcemia, skeletal deformities, and potentially cardiogenic shock.
Purpose of the Study:
- To report a case of pediatric DCM secondary to vitamin D-deficient rickets.
- To highlight the diagnostic challenges and management of this rare condition.
- To emphasize the potential for cardiac recovery with timely intervention.
Main Methods:
- Case report of a 16-month-old male with DCM and cardiogenic shock.
- Diagnostic workup included laboratory tests for hypocalcemia and vitamin D levels, and radiographic skeletal surveys.
- Management involved mechanical circulatory support with venoarterial extracorporeal membrane oxygenation (VA ECMO) and a left ventricular assist device (LVAD), alongside aggressive vitamin D and calcium repletion.
Main Results:
- The patient presented with severe left ventricular dysfunction, hypotension, and hypocalcemia.
- Diagnosis of vitamin D-deficient rickets was confirmed by low vitamin D levels and characteristic radiographic findings.
- Despite initial aggressive treatment, VA ECMO was required, followed by LVAD support. Cardiac function gradually normalized, allowing for device explantation after 95 days.
- The patient recovered normal left ventricular function at 1-year follow-up, despite a stroke during LVAD support.
Conclusions:
- Vitamin D-deficient rickets is a rare but reversible cause of pediatric DCM.
- Left ventricular assist device (LVAD) support can serve as a bridge to recovery in severe rickets-associated DCM.
- Early recognition and comprehensive management are crucial for improving outcomes in these patients.
Abstract:
Dilated cardiomyopathy (DCM) in pediatric patients is most commonly of idiopathic, genetic, or infectious etiology, but rare cases are reported secondary to vitamin D-deficient rickets. Mild rickets is typically associated with skeletal deformities, delayed gross motor milestones, and growth failure, while severe presentations may involve hypocalcemic seizures or DCM. We report a 16-month-old male patient presenting with lethargy, tachypnea, hypotension, and hepatomegaly who was found to have severe left ventricular dysfunction and associated cardiogenic shock. Initial management included intubation, inotropic support, and cannulation to venoarterial extracorporeal membrane oxygenation (VA ECMO). He had hypocalcemia on presentation, laboratory studies demonstrating a low vitamin D level, and radiograph findings of rachitic rosary, splaying of distal metaphyses, and generalized demineralization leading to a diagnosis of vitamin D-deficient rickets. Despite aggressive vitamin D and calcium repletion, the patient was unable to wean from VA ECMO, ultimately requiring transition to a Berlin Heart EXCOR pediatric left ventricular assist device (LVAD) on day 20 of hospitalization. The patient's hospital course was complicated by an LVAD-associated thromboembolic stroke with mild residual neurologic deficits after an unsuccessful mechanical thrombectomy. His left ventricular function gradually normalized, leading to successful explantation after 95 total days of mechanical support. The patient's left ventricular function remained normal at 1 year follow-up. This case highlights a rare but reversible cause of pediatric DCM and includes the use of LVAD support as a bridge to recovery in rickets-associated DCM.
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