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Pulmonary artery banding in infants and young children with left ventricular dilated cardiomyopathy: A novel therapeutic strategy before heart transplantation

https://doi.org/10.1016/j.healun.2013.01.988Get rights and content

Background

Dilated cardiomyopathy (DCM) in childhood has a considerable morbidity and mortality and high incidence of heart transplantation. Pulmonary artery banding (PAB) has been proposed in patients with corrected transposition of the great arteries to retrain the sub-pulmonic left ventricle (LV) and to improve a failing sub-aortic right ventricle. We evaluated the short-term and medium-term effects of PAB in young patients with LVDCM.

Methods

A retrospective single-center observational study was performed to evaluate the possible benefits of a dilatable surgical PAB in infants and young children with LVDCM.

Results

Reported are 12 patients (10 infants, 2 toddlers) with LVDCM referred for heart transplant who received a surgical PAB. There were no hospital deaths. Clinical functional status improved in all patients. The pressure gradient across the PAB increased within 20 days from 28 ± 7 to 43 ± 15 mm Hg. The LV ejection fraction increased from 14.5% ± 5% pre-PAB to 27% ± 13% at hospital discharge and to 47% ± 10% at 3 to 6 months. The LV end-diastolic diameter (z-score) decreased (p > 0.001) from 46 ± 6.1 (+7.0 ± 1.3) to 35 ± 15 mm (+3.0 ± 1.3) after 3 to 6 months and to 34 ± 15 mm (+1.3 ± 1.14) after a median age of 2 years (maximum 6.6 years), respectively. Plasma B-type natriuretic peptide levels decreased from 3431 ± 2610 to 288 ± 321 pg/ml at discharge and to 102 ± 96 pg/ml 22 months later. Eight children were subsequently de-banded by transcatheter technique and 6 of them are currently at Ross Heart Failure Classification for Children class I. Two patients, both with non-compaction DCM, deteriorated at 5 and 6 months after PAB debanding and finally died.

Conclusion

In young children with LVDCM and still-preserved right ventricular function, PAB led to an improvement of LV and mitral valve function by ventricular interaction.

Section snippets

Patients and methods

From April 2006 to December 2012, 56 patients aged younger than 3 years old were referred to our Pediatric Heart Center because of dilative LV heart failure. Of these, 17 received a surgical PAB. In 5 of the 17 patients with LVDCM, a PAB was performed in addition to open heart surgery for repair of a left-sided anomalous pulmonary venous return, large atrial septal defect by fenestrated patch, reimplantation of an anomalous origin of the left coronary artery from the pulmonary artery, and

Results

PAB was successfully performed in all patients. There were no hospital deaths. Most valuable clinical, echocardiographic, MRI and BNP data before and after PAB are presented as single values of each patient and summarized in Table 1 , Table 2 .

After an individualized tightening of the PAB, a systolic RV pressure as a level of 60% to 70% of the systolic arterial blood pressure was achieved in all patients. The pressure gradient across the PAB increased significantly (p > 0.01) from 28 ± 7 mm Hg

Discussion

Common unfavorable outcomes of DCM are death, usually resulting from congestive heart failure or sudden cardiac death, and HTx. Reported freedom from death and HTx are 72% and 69% at 1 year and 63% and 54% at 5 years.1, 12 Survival rates from the time of the DCM diagnosis have been unchanged for several decades.13, 14 Thus, there is a need to pursue additional strategies to avoid or to delay HTx in the pediatric DCM population. We hypothesized that PAB might serve as such a novel therapeutic

Disclosure statement

None of the authors has a financial relationship with a commercial entity that has an interest in the subject of the presented manuscript or other conflicts of interest to disclose.

References (18)

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