Thorac Cardiovasc Surg 2019; 67(S 02): S101-S128
DOI: 10.1055/s-0039-1679046
Oral Presentations
Sunday, February 17, 2019
Kinderkardiologische/Kinderkardiochirugische Intensivmedizin
Georg Thieme Verlag KG Stuttgart · New York

Combined Surgical and Medical Approach in Left Ventricular Dilated Cardiomyopathy for Restoring Synchrony and Ventricular–Ventricular Interaction

S. Recla
1   Universitätsklinik Gießen u. Marburg Standort Gießen, Giessen, Germany
,
B. Steinbrenner
1   Universitätsklinik Gießen u. Marburg Standort Gießen, Giessen, Germany
,
L. Rueblinger
1   Universitätsklinik Gießen u. Marburg Standort Gießen, Giessen, Germany
,
T. Logeswaran
1   Universitätsklinik Gießen u. Marburg Standort Gießen, Giessen, Germany
,
S. Skrzypek
1   Universitätsklinik Gießen u. Marburg Standort Gießen, Giessen, Germany
,
K. Gummel
1   Universitätsklinik Gießen u. Marburg Standort Gießen, Giessen, Germany
,
B. Reich
1   Universitätsklinik Gießen u. Marburg Standort Gießen, Giessen, Germany
,
D. Schmidt
1   Universitätsklinik Gießen u. Marburg Standort Gießen, Giessen, Germany
,
J. Thul
1   Universitätsklinik Gießen u. Marburg Standort Gießen, Giessen, Germany
,
M. Mueller
1   Universitätsklinik Gießen u. Marburg Standort Gießen, Giessen, Germany
,
C. Jux
1   Universitätsklinik Gießen u. Marburg Standort Gießen, Giessen, Germany
,
H. Akintürk
1   Universitätsklinik Gießen u. Marburg Standort Gießen, Giessen, Germany
,
D. Schranz
1   Universitätsklinik Gießen u. Marburg Standort Gießen, Giessen, Germany
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Publikationsverlauf

Publikationsdatum:
28. Januar 2019 (online)

Objectives: Left ventricular dilated cardiomyopathy (LV-DCM) is characterized by a dilation and dysfunction of the left ventricular chamber. The incidence of LV-DCM has been reported 0.57–1.13/100,000 and remains a leading cause of cardiac death in children. Approximately 40% of children die or need cardiac transplantation in the first 5 years after diagnosis. The reported combined medical and surgical approach represents a novel therapeutic strategy with the aim to restore ventricular–ventricular interaction (VVI) and induce myocyte recovery.

Methods: Thirty-two patients below the age of 3 years with an end-stage DCM and Ross functional status III–IV were referred to our center for heart transplantation and received, as additional measure, a surgical pulmonary artery banding (PAB). With the hypothesis, that a reversible PAB combined with anticongestive therapy can avoid or defer a cardiac transplantation, the efficiency of this therapy was retrospectively analyzed with the use of clinical, laboratory-chemical, and imaging data.

Results: All patients survived surgical intervention to discharge. Follow-up after 38 ± 28 months showed an improvement of clinical functional status (Ross I, Ross II) and a significant improvement, up to normalization, of the analyzed data (expressed in mean ± standard deviation): left ventricular ejection fraction (LV-EF) increased from 17 ± 6 to 58 ± 8%, left ventricular end-diastolic diameter (LVEDD) decreased from 46 ± 5 to 35 ± 5 mm (z-score from +6.9 ± 1.3 to +0.9 ± 0.8), BNP decreased from 3,089 ± 2,642 to 64 ± 82 ng/L. Sixteen patients have undergone catheter-based debanding after a median of 10.5 months.

After 3 years, left ventricular function normalized in 81% of patients, who were free from transplantation.

Conclusion: VVI plays a key role in the human heart. We suppose that PAB in LV-DCM restores the LV-synchrony by shifting the ventricular septum leftward and thereby reducing left ventricular end-diastolic volume and pressure. Restored VVI and LV-geometry, the PAB-induced pressure overload of the right ventricle and the specific anticongestive therapy, stimulate all together the endogenous regenerative ability of the human heart. Yet, further questions concerning cellular mechanism of action, optimal time point for intervention, and identification of subgroups with risk for treatment failure still need to be examined.