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Diagnosed as dilated cardiomyopathy 10 years ago.
8 years ago ICD was implanted and his symptoms improved with GDMT.
Recently aggravated dyspnea on exertion with persistently low LVEF.
Clinical examination
BP 94/60 mmHg
Heart rate 86/min
S3 (+)
JVP elevated
Laboratory test results
E’ Na 138 mmol/L K 4.0 mmol/L
BUN 13.9 mg/dL
Cr 0.7 mg/dL
Hb 16.6 g/dL
NT-proBNP 3120 pg/mL
TTE and MRI images
Echo CG LVEF of 17% severe global hypokinesia, mild to moderate MR
CAG
Normal Coronary angiogram with ICD lead in place
CXR
Chest PA view shows cardiomegaly, pulmonary congestion, ICD in situ
ECG
ECG showing Normal sinus rhythm, Bi-atrial enlargement, RBBB, VPC
He was diagnosed as having advanced heart failure due to dilated cardiomyopathy and his medication of ACE inhibitor was replaced by Sacubitril/Valsartan 100 mg bid and Ivabradine was added.
After adjustment of medications his NT-proBNP level was reduced to the level of 1443 pg/mL with slight improvement of symptoms.
However, after 18 months of management his symptom re-aggravated with persistent elevation of NT-proBNP up to 3592 pg/mL. There was no evidence of arrhythmia, infection, anemia, or thyroid dysfunction.
He also denied any other aggravating factors such as high salt diet, skipped medication, or medicines such as NSAID. This was second admission due to aggravation of HF since 6 months before.
Now he is referred to the advanced HF clinic if he is eligible to HF surgery such as LVAD and/or heart transplantation.
Author’s opinion
As up-to-date guideline-directed medical treatment could not improve his symptom and his NT-proBNP level is persistently high
above 3000 pg/mL, he can be a candidate for surgical treatment of HF such as LVAD and/or heart transplantation.
He would undergo evaluation such as right heart catheterization and exercise test measuring peak oxygen consumption rate. The tests would confirm if he is eligible for LVAD surgery or heart transplantation.
Domingo A. Pascual-Figal. et al, European Hear Journal, Volume 29, Issue 8, April 2008, Pages 1011-1018
61-year old woman
Key signs and symptoms
Recent aggravation of DOE with exertional chest pain
Medical History
1 year ago dyspnea on exertion fc 2 Recently aggravated as NYHA III with exertional chest pain
Mild concentric LVH Normal LV function
Normal RV function
Diagnosed for subglottic cancer previously but currently no evidence of recurrence
Suggested for further evaluation
Clinical examination
BP 91/63 mmHg
Heart rate 63/min
Pretibial pitting edema (+/+)
In case of HFpEF, if patient has very high NT-proBNP level, normal kidney function and sinus rhythm, think about cardiac amyloid.
This patient was diagnosed as having primary cardiac amyloidosis was confirmed by cardiac biopsy together with bone marrow examination.
Author’s opinion
Echocardiogram gives a hint of amyloid if read by an experienced person, but the initial diagnosis did not include amyloidosis.
Level of NT-proBNP, its level of > 3000 pg/mL in the case with normal EF, sinus rhythm and no significant renal dysfunction, one should think about cardiac amyloid. 1
And in the case of normal coronary angiogram for typical angina chest pain, it is recommended to measure LVEDP.
Elevated LVEDP, very high NT-proBNP, mildly elevated cardiac troponin, without evidence of renal failure could be a clue to the diagnosis of cardiac amyloidosis with normal coronary angiogram.
This patient was diagnosed as having primary cardiac amyloidosis was confirmed by cardiac biopsy together with bone marrow examination and was treated with chemotherapy for plasma cell dyscrasia.
약물 조정 후, NT-proBNP 수치는 1443 pg/mL 수준으로 감소했으며 증상도 약간 호전되었습니다.
그러나 18개월 관리 후, NT-proBNP가 최대 3592 pg/mL까지 지속적으로 상승하고 증상이 재악화되었습니다. 부정맥, 감염, 빈혈 또는 갑상선 기능장애의 증거는 없었습니다.
또한 환자는 고염식, 약물 복용 누락 또는 NSAID 등의 약물과 같은 다른 악화 요인들을 차단했습니다. 이는 6개월 전부터 HF 악화로 인한 두 번째 입원이었습니다.
이제 환자가 좌심실 보조장치(left ventricular assist device, LVAD) 및/또는 심장 이식과 같은 심부전 수술에 적합한 경우, 진행성 심부전 클리닉에 환자를 의뢰합니다.
저자 의견
최신 가이드라인에 따른 의학적 치료로 증상이 호전되지 않았고 지속적으로 3000 pg/mL이 넘는 높은 NT-proBNP 수치가
나타나므로, 환자는 LVAD 및/또는 심장 이식과 같은 심부전의 외과적 치료에 적합한 후보일 수 있습니다.
환자는 우심도자술, 최고 산소 소비 속도를 측정하는 운동 검사와 같은 평가를 받을 것입니다. 이 검사는 환자가 LVAD 수술 또는 심장 이식에 적합한지 확인할 것입니다.
Domingo A. Pascual-Figal. et al, European Hear Journal, Volume 29, Issue 8, April 2008, Pages 1011-1018
61세 여성
주요 징후 및 증상
운동성 흉통을 동반한 운동시 호흡곤란(Dyspnea on exertion, DOE)이 최근 악화됨
병력
1년전 운동 시 호흡곤란 fc 2 운동성 흉통을 동반한 NYHA 3등급으로 최근 악화
경증 동심 좌심실비대(left ventricular hypertrophy, LVH) 정상 좌심실 기능
정상 우심실 기능
이전 성문하암을 진단받았으나 현재 재발의 증거 없음
추가 평가가 제안됨
임상 검사
BP 91/63 mmHg
심박수 63/분
경골전 오목 부종(+/+)
실험실 검사 결과
Hb 14.7 g/dL
E’ Na 143 mmol/L
K 3.9 mmol/L
BUN 16 mg/dL
Cr 1.19 mg/dL
NT-proBNP 4430 pg/mL
TnI 0.059 ng/mL
혈청 유리 Kappa 경쇄 474 mg/L
혈청 유리 Lambda 경쇄 6.18 mg/L
