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Phantoms of the Past: Multiple Organ Dysfunction in a Patient with Tetralogy of Fallot and Relapse of Diamond-Blackfan Anemia
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A3462 - Phantoms of the Past: Multiple Organ Dysfunction in a Patient with Tetralogy of Fallot and Relapse of Diamond-Blackfan Anemia
Author Block: Z. Imam, Y. Pang, D. T. Keena; William Beaumont Hospital-Royal Oak, Royal Oak, MI, United States.
Introduction:
Adult intensivists have increasing exposure to individuals with congenital diseases surviving into adulthood. Solid knowledge bases and early recognition of the possible sequelae of congenital disorders are crucial in the care of these patients. We present a challenging case of acute heart failure exacerbation and severe anemia due to the relapse of Diamond-Blackfan anemia, and complications arising from congenital heart disease.
Case presentation:
A 42-year-old man with Tetralogy of Fallot (TOF), and Diamond-Blackfan anemia (DBA) presented with worsening dyspnea and fatigue. He denied fever, chest pain, melena, hematochezia, or hematuria. He underwent repair of TOF in infancy and was treated with systemic steroids for DBA with achievement of remission. The patient had been lost to medical follow-up since the age of 24.
He was pale and was found to be in circulatory shock with hypotension, tachycardia, and respiratory distress. He had a Klippel-Feil deformity and a triphalangeal right thumb. A harsh diastolic murmur was auscultated over the left sternal border. Labs showed a hemoglobin of 2.5 g/dl, WBC count of 26.4/mm3, platelet count of 188/mm3, lactic acid of 19 mmol/L, AST 1,072 U/L, ALT 1,568 U/L, INR 3.2, and a procalcitonin of 6.06 ng/ml. BNP was 2942 pg/ml. Chest X-ray revealed cardiomegaly, bilateral interstitial infiltrates, and opacification of the left lower lobe. Echocardiography showed severe enlargement of the right ventricle. He was given packed RBC transfusions, broad-spectrum antibiotics, and vasopressors. Despite non-invasive positive pressure ventilation measures, his hypoxic respiratory failure worsened, requiring intubation. After three days of ventilator support, diuresis, and empiric antibiotics, his respiratory and heart failure improved. Lactic acid, transaminases, and INR normalized. He was successfully extubated. No other etiology of anemia was identified, and the patient was started on prednisone for relapsed DBA.
Conclusion:
Approximately 15% of post-TOF repair patients develop pulmonary hypertension with right ventricular dilatation and dysfunction. In our case, a high-output status created by severe anemia triggered biventricular decompensation with subsequent pulmonary edema and ischemic hepatitis. Despite the successful treatment of this patient, we cannot ignore the psychological and physical morbidity of invasive procedures and intensive care unit stay, which might have been prevented by early detection of the DBA relapse and right ventricular failure. 61% of patients with congenital heart disease are lost to follow up after age 18. Health care systems will be increasingly challenged to develop new strategies to successfully transition complex pediatric patients to adult care and maintain continuity.
Author Block: Z. Imam, Y. Pang, D. T. Keena; William Beaumont Hospital-Royal Oak, Royal Oak, MI, United States.
Introduction:
Adult intensivists have increasing exposure to individuals with congenital diseases surviving into adulthood. Solid knowledge bases and early recognition of the possible sequelae of congenital disorders are crucial in the care of these patients. We present a challenging case of acute heart failure exacerbation and severe anemia due to the relapse of Diamond-Blackfan anemia, and complications arising from congenital heart disease.
Case presentation:
A 42-year-old man with Tetralogy of Fallot (TOF), and Diamond-Blackfan anemia (DBA) presented with worsening dyspnea and fatigue. He denied fever, chest pain, melena, hematochezia, or hematuria. He underwent repair of TOF in infancy and was treated with systemic steroids for DBA with achievement of remission. The patient had been lost to medical follow-up since the age of 24.
He was pale and was found to be in circulatory shock with hypotension, tachycardia, and respiratory distress. He had a Klippel-Feil deformity and a triphalangeal right thumb. A harsh diastolic murmur was auscultated over the left sternal border. Labs showed a hemoglobin of 2.5 g/dl, WBC count of 26.4/mm3, platelet count of 188/mm3, lactic acid of 19 mmol/L, AST 1,072 U/L, ALT 1,568 U/L, INR 3.2, and a procalcitonin of 6.06 ng/ml. BNP was 2942 pg/ml. Chest X-ray revealed cardiomegaly, bilateral interstitial infiltrates, and opacification of the left lower lobe. Echocardiography showed severe enlargement of the right ventricle. He was given packed RBC transfusions, broad-spectrum antibiotics, and vasopressors. Despite non-invasive positive pressure ventilation measures, his hypoxic respiratory failure worsened, requiring intubation. After three days of ventilator support, diuresis, and empiric antibiotics, his respiratory and heart failure improved. Lactic acid, transaminases, and INR normalized. He was successfully extubated. No other etiology of anemia was identified, and the patient was started on prednisone for relapsed DBA.
Conclusion:
Approximately 15% of post-TOF repair patients develop pulmonary hypertension with right ventricular dilatation and dysfunction. In our case, a high-output status created by severe anemia triggered biventricular decompensation with subsequent pulmonary edema and ischemic hepatitis. Despite the successful treatment of this patient, we cannot ignore the psychological and physical morbidity of invasive procedures and intensive care unit stay, which might have been prevented by early detection of the DBA relapse and right ventricular failure. 61% of patients with congenital heart disease are lost to follow up after age 18. Health care systems will be increasingly challenged to develop new strategies to successfully transition complex pediatric patients to adult care and maintain continuity.
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