Yonsei Cardiovascular Center

Presentation on theme: "Yonsei Cardiovascular Center"— Presentation transcript:

1 Yonsei Cardiovascular Center
CASE Yonsei Cardiovascular Center

2 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

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

4 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

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

6 Chest PA (HD #1)

7 ECG (HD #1)

8 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

9 Abdomen CT (HD #1)

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

11 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

12 Abdomen CT F/U (HD #14)

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

14 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

15 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

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

17 HD #22, PD #6

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

19 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

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