(II-1B1) Spectacular improvement in a 50 year old urgent heart transplant candidate after 3-month phosphodiesterase type 5 inhibitor (sildenafil) treatment Marta Szuksztul, Paweł Rubiś MD, PhD, Monika Dzieciuch-Rojek, Sylwia Wiśniowska-Śmiałek MD, Dawid Kudliński, Agata Leśniak-Sobelga MD, PhD, Magdalena Kostkiewicz MD, PhD, Prof. Piotr Podolec MD, PhD



EXPERT: Prof. Roland Hetzer, MD, PhD
Affiliation: German Heart Instiute, Berlin, Germany
The patient responded well to the administered treatment (improvement of LVEF and gradual reduction of pulmonary hypertension) although the LV size didn’t change. Due to severe mitral regurgitation mitral valve repair should be considered, cardiosurgical consultation is required. The patient may need heart transplantation in the future. Furthermore CRT may be an option if such a procedure is technically feasible.

EXPERT: Prof. Egle Ereminiene, MD, PhD
Affiliation: Hospital of Lithuanian University of Health Sciences, Kaunas,
The patient’s condition has improved and therefore the decision on heart transplantation should be postponed. As to management of severe mitral regurgitation mitral-clip is not recommended (mitral annulus is large). It would be interesting to measure effect of sildenafil treatment on the coexistent combined aortic valve disease if mitral valve repair operation would be indeed performed.

EXPERT: Prof. Lesław Szydłowski, MD, PhD
Affiliation: 1st Department of Paediatric Cardiology, Medical University of Silesia, Katowice-Ligota, Poland
It is probable that the primary pathology in this patient was restrictive cardiomyopathy (the atria are large). It is debatable if the patient’s condition and LV function improved owing solely to sildenafil treatment or due to effective standard medication therapy of congestive heart failure (or perhaps it was a combined effect of both).

EXPERT: Grzegorz Kopeć, MD, PhD
Affiliation: Department of Cardiac and Vascular Diseases, John Paul II Hospital, Krakow, Poland
Sildenafil therapy in patients with dilated cardiomyopathy and heart failure is not a standard approach and requires further studies. In the presented case the drug lowered both pulmonary and systemic vascular resistance thus perhaps improving the LVEF. It is notable that no other modifications except sildenafil introduction were made.

EXPERT: Assoc. Prof. Bogusław Kapelak, MD, PhD
Affiliation: Department of Cardiovascular Surgery and Transplantology, John Paul II Hospital, Krakow, Poland
As to optimal cardiosurgical management the patient would probably benefit more from heart transplantation than mitral valve repair. Personal experience with undersizing mitral annuloplasty has shown that in patients with large LV size results of the operation are transient and do not improve LV function – after 2-3 years those patients eventually have to undergo HTx .


1. At present optimal pharmacotherapy appears to be the treatment of choice. Heart transplantation is indicated if the patient’s condition worsens.
2. Electrocardiological consultation is recommended – the patient may be a candidate for cardiac resynchronization therapy.
3. If mitral valve repair is to be considered (preferably undersizing mitral annuloplasyty) the aortic valve disease should be precisely assessed before such an operation.
4. The patient should remain under strict medical observation.

“Development of the European Network in Orphan Cardiovascular Diseases”
„Rozszerzenie Europejskiej Sieci Współpracy ds. Sierocych Chorób Kardiologicznych”

This entry was posted in 1. Pulmonary hypertension, 1. Severe pulmonary hypertension due to left heart diseases, B. Severe forms of non-low-prevalence pulmonary hypertension, Case presentations, II. Rare diseases of pulmonary circulation. Bookmark the permalink.

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