- Non-smoker
- No alcohol consumption
- Blood pressure: 92/61 mmHg
- Heart rate: 101
- Cardiac auscultation revealed S1, S2 heart sounds with S3 and a holosystolic murmur by left parasternal border.
- Bilateral pretibial edema was present (+3).
- BNP:4500 pg/ml, creatinine:1.3 mg/dl, potassium:4.8 pg/ml
- ECG: 2nd day Electrocardiogram demonstrated sinus rhythm (SR) with a heart rate (HR) of 82 beats per minute with signs of atrial dilatation.
- Chest CT: Thoracic computed tomography (CT) revealed an increased cardiothoracic ratio. The pulmonary trunk diameter was enlarged at 35 mm. The trachea and main bronchi were patent. There was an increase in the amount of pleural effusion on the left hemithorax, measuring approximately 2 cm. Interlobular septal thickening was noted bilaterally. Additionally, peripheral predominant ground-glass opacities and consolidations were observed in the right lung, consistent with the findings on the patient’s previous imaging.
- Left ventricular ejection fraction (LVEF): 15% (global hypokinesia with spontaneous echo contrast within left ventricle)
- Mitral regurgitation: Moderate (EROA 0.19 cm²)
- Tricuspid regurgitation: Severe (grade 3)
- Systolic pulmonary artery pressure: 50–55 mmHg
- No regional wall motion abnormalities observed in the left ventricle
- Hepatic veins and inferior vena cava (IVC) diameter 2.1 cm with reduced respiratory collapse
- Left atrium diameter: 5.5 mm
- Lead from ICD appropriately positioned in the dilated right heart structures
- New York Heart Association (NYHA) Class III symptoms
- LVEF <30%
- Elevated NT-proBNP levels
- Requirement for high-dose intravenous diuretics
- NYHA Class III symptoms
- LVEF below 20%
- More than one heart failure hospitalization within the last year
- Persistent fluid overload and escalating diuretic requirement
- Persistent low systolic blood pressure (<90–100 mmHg)
- Inability to tolerate or uptitrate guideline-directed medical therapy
A 36-year-old male patient who had been diagnosed as Becker muscular dystrophy (BMD) 25 years ago, presented with acute decompensated heart failure (ADHF), having had three hospitalizations in the last three months requiring high doses of diuretics. The patient reported a one-month history of progressively worsening exertional dyspnea and bilateral lower extremity edema.
Medical history
The patient’s past medical history was notable for Becker muscular dystrophy along with compatible physical findings, i.e, significant proximal muscle atrophy and calf pseudohypertrophy. Hence, he was bound by a wheelchair.
He also suffered from deep vein thrombosis, and pulmonary thromboembolism in 2018 in relation to immobility.
He was diagnosed to have heart failure in 2006 and underwent primary prophylaxis with a St. Jude implantable cardioverter-defibrillator (ICD-VVI) in 2016.
There is no history of coronary artery disease.
History was remarkable for several on and off in relation to HF medications along with low adherence due to low blood pressure, which has become more apparent in recent years.
Cardiac status
Echocardiographic findings were consistent with heart failure with reduced ejection fraction (HFrEF), characterized by a dilated left ventricle.
Social history
Investigation on admission
12-lead ECG of the patient at admission
Transthoracic echocardiography (TTE)
Echocardiography wtih 4-chamber view and continuous Doppler at mitral valve
Video of 4-chamber view echocardiography
Clinical Assessment
The patient, along with his history in the last year is consistent with the European Society of Cardiology (ESC) 2021 guidelines criteria for advanced heart failure, including:
Additionally, the patient met several markers of poor prognosis defined by the AHA’s 'I NEED HELP' criteria1, associated with an estimated one-year all-cause mortality rate of approximately 46.5%:
- Theresa A McDonagh, Marco Metra, Marianna Adamo, Roy S Gardner, Andreas Baumbach, Michael Böhm, Haran Burri, Javed Butler, Jelena Čelutkienė, Ovidiu Chioncel, John G F Cleland, Andrew J S Coats, Maria G Crespo-Leiro, Dimitrios Farmakis, Martine Gilard, Stephane Heymans, Arno W Hoes, Tiny Jaarsma, Ewa A Jankowska, Mitja Lainscak, Carolyn S P Lam, Alexander R Lyon, John J V McMurray, Alexandre Mebazaa, Richard Mindham, Claudio Muneretto, Massimo Francesco Piepoli, Susanna Price, Giuseppe M C Rosano, Frank Ruschitzka, Anne Kathrine Skibelund, ESC Scientific Document Group , 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure: Developed by the Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC) With the special contribution of the Heart Failure Association (HFA) of the ESC, European Heart Journal, Volume 42, Issue 36, 21 September 2021, Pages 3599–3726, https://doi.org/10.1093/eurheartj/ehab368
- Gyoten T, Amiya E, Kinoshita O, Tsuji M, Kimura M, Hatano M, Ono M. Clinical outcomes of continuous flow left ventricular assist device therapy as bridge to transplant strategy in muscular dystrophy: a single-center study. Gen Thorac Cardiovasc Surg. 2023 Jun;71(6):347-353. doi: 10.1007/s11748-022-01889-1. Epub 2022 Nov 8. PMID: 36348232.
- Skouri H, Girerd N, Monzo L, Petrie MC, Böhm M, Adamo M, Mullens W, Savarese G, Yilmaz MB, Amir O, Bayes-Genis A, Bozkurt B, Butler J, Chioncel O, Mebazaa A, Merino JL, Moura B, Ponikowski P, Seferovic P, Rosano GMC, Metra M. Clinical management and therapeutic optimization of patients with heart failure with reduced ejection fraction and low blood pressure. A clinical consensus statement of the Heart Failure Association (HFA) of the ESC. Eur J Heart Fail. 2025 Apr;27(4):707-722. doi: 10.1002/ejhf.3618.
- Mustafa Goktug Erata: None
- Mehmet Birhan Yilmaz: has received institutional fee from Bayer, Amgen, Novartis, Astra Zeneca, Boehringer Ingelheim, Novo Nordisk, Albert Health
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