Yonsei Cardiovascular Center CASE Yonsei Cardiovascular Center 5313922

15-year-old boy Chief Complaint: • Aggravated dyspnea on exertion for 3 days • Referred from a private clinic for evaluation of documented abdominal mass & LV dysfunction on ultrasonogram

Past History: Hypertension (–) DM (–) Alcohol (–) Family History: None Review of System: Dyspnea on exertion (+) Palpitation / Chest pain (-/-) Headache (-) Abdominal pain (-)

Physical Examination: BP 114 / 70 mmHg PR 128 bpm Distended Jugular vein Crackles on both lower lobes RHB without murmurs No Hepatomegaly or splenomegaly No pitting edema on both extremites

Laboratory findings: CBC 9740 / 15.6 / 248,000 BUN / Cr 22.7 / 1.1 mg/dL Glucose 102 mg/dL U/A Pro (-) Glu (-) Pro / Alb 6.0 / 4.2 g/dL AST / ALT 364 / 428 IU/L T.Bil / D.Bil 1.7 / 1.1 mg/dL CK / CK-MB / Tn-T 50 / 5.01 / 0.012 NT-proBNP 17,402 pg/mL

Chest PA (HD #1)

ECG (HD #1)

Echocardiography (HD #1) 1. Severely depressed LV systolic function (EF=13%) with multiple thrombi in LV apex 2. Severe hypokinesia of RV 3. Enlarged LA (volume index=34cc/m2) and borderline enlarged LV 4. Mild pulmonary hypertension (RVP=42mmHg), dilated IVC with plethora

Abdomen CT (HD #1)

• Epinephrine 39.8 ug/day (0~20) 24 hr urine study: • Epinephrine 39.8 ug/day (0~20) • Metanephrine 11.1 mg/day (0~1.3) • Norepinephrine 10,531 ug/day (15~80) • VMA 34.1 mg/day (0~8)

DIAGNOSIS: Catecholamine induced cardiomyopathy LV thrombi, multiple Pheochromocytoma Captopril (ACE inhibitor), Heparin infusion, Doxazocin (alpha-blocker) 투여 시작 입원 14일째 갑작스런 복통 호소하여 CT F/U 하였으며, Pheochromocytoma rupture 의심되었으며, 입원 16일째 right adrenalectomy 시행함

Abdomen CT F/U (HD #14)

Adrenalectomy, Right (HD #16) Adrenal Gland 37.9 g, 6.5 x 4.5 x 4 cm x12 x100

PROGRESS 114/70 mmHg 100/70 mmHg 42/21 mmHg 128 bpm 130 bpm 140 bpm HOD#1 HOD#8 #14 #16 #18 #20 Rupture of Pheochromocytoma PCPS CRRT Adrenalectomy, Right 114/70 mmHg 128 bpm 100/70 mmHg 130 bpm 42/21 mmHg 140 bpm Heparin Heparin Captopril 25mg tid Doxazosin 1mg bid 2mg bid Phenoxybenzamine 10mg qd Aldactone 12.5mg qd Ciprofloxacin 200mg bid, Tazocin 4.5g tid Epinephrine 0.3ug/min/Kg Norepinephrine 85ug/min Vasopression 0.12ug/min/Kg

수술 후 2 일째 흉부 단순 촬영에서 폐부종 악화, 혈압 및 산소포화도 저하되면서 심정지 발생하여 15분간 흉부 압박 후 자발순환 회복되었고, 심기능 저하, 폐부종 악화되어 심폐보조순환술 시행 수술 후 4 일째 소변량 감소하고 산혈증 진행되어 지속적정정맥혈액투석 시행하였으나 폐부종 악화되어 혈압 및 산소포화도 유지되지 않았으며 동공반사 소실되어 매우 불량한 예후 예상되었음. 당시 Ejection fraction 5% 로 회복소견 보이지 않았음 입원 22 일째 맥박 소실, 심전도상 정지 소견 보여 사망선언

After Operation After PCPS HOD #17, POD #1 HOD #19, POD #3

HD #22, PD #6

Cardiac manifestations of Pheochromocytoma Cardiomyopathies Forms of myocardial ischemia Primarily arrhythmogenic conditions

Mechanism of Catecholamine induced Cardiomyopathy Catecholamine-induced vasomotor constriction of the myocardial circulation Catecholamine direct toxicity Calcium overload Catecholamine oxidation product   Free Radical  Sarcolemmal permeability Ischemic damage Myocardial change Focal areas of myocytolysis Myofiber necrosis Interstitial fibrosis Mononuclear inflammatory infiltrates

Catecholamine-induced cardiomyopathy can be irreversible or reversible. In the presence of heart failure or pulmonary edema, one must consider pheochromocytoma as the cause.