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Hybrid muscular ventricular septal defect closure in a 4.5 kg infant followed by sildenafil treatment and transcatheter atrial septal defect occlusion

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112 Advances in Interventional Cardiology 2018; 14, 1 (51)

Image in intervention

Corresponding author:

Michal Galeczka MD, Department of Congenital Heart Defects and Pediatric Cardiology, SMDZ in Zabrze, Medical University of Silesia, Silesian Center for Heart Diseases, 9 Marii Curie-Sklodowskiej St, 41-800 Zabrze, Poland, phone: +48 609 345 681, e-mail: michalgaleczka@gmail.com Received: 12.11.2017, accepted: 10.12.2017.

Hybrid muscular ventricular septal defect closure in a 4.5 kg infant followed by sildenafil treatment and transcatheter atrial septal defect occlusion

Michal Galeczka1, Roland Fiszer1, Szymon Pawlak2, Joanna Sliwka2, Linda Litwin1, Malgorzata Szkutnik1

1 Department of Congenital Heart Defects and Pediatric Cardiology, SMDZ in Zabrze, Medical University of Silesia, Silesian Center for Heart Diseases, Zabrze, Poland

2 Department of Cardiac, Vascular and Endovascular Surgery and Transplantology, SMDZ in Zabrze, Medical University of Silesia, Silesian Center for Heart Diseases, Zabrze, Poland

Adv Interv Cardiol 2018; 14, 1 (51): 112–114 DOI: https://doi.org/10.5114/aic.2018.74368

A  4.5 kg 3-month-old girl was admitted with heart failure symptoms. Transthoracic echocardiography (TTE) revealed 5.5 mm midmuscular ventricular septal defect (VSD) with bidirectional shunt, 6 mm typically located secundum atrial septal defect (ASD), right heart enlarge- ment and signs of pulmonary hypertension (PH). Our heart team decided to perform a hybrid VSD closure. Af- ter sterno- and pericardiotomy and heart apex elevation the right ventricle (RV) was punctured on the beating heart under epicardial echocardiography (EE) guidance

(Figure 1). Right ventricle pressure of 40/0/6 mm Hg and arterial pressure of 64/40/50 mm Hg were measured.

The VSD was crossed with a  J-tip guidewire, and then a  8 French (Fr) delivery sheath was advanced. Taking into consideration the delicate manual maneuvers, the 7 mm Hyperion VSD Muscular Occluder (Comed B.V., The Netherlands/Lepu MT Company, China) was successfully deployed under EE (Figure 2) – an insignificant residu- al leak was observed. The intervention was uneventful.

On the first day after the procedure during weaning from

Figure 1. Epicardial echocardiography. Ventricular septal defect (VSD) – asterisk

RV – right ventricle, LV – left ventricle.

Figure 2. Epicardial echocardiography. 7 mm Hy- perion VSD Muscular Occluder deployed in VSD over delivery cable

RV – right ventricle, LV – left ventricle.

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Michal Galeczka et al. Hybrid approach for muscular VSD and ASD

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Advances in Interventional Cardiology 2018; 14, 1 (51)

mechanical ventilation a  pulmonary hypertensive crisis occurred, manifested by significant bradycardia and ar- terial saturation fall. Therefore, NO inhalation, sildenafil (15 mg/day), milrinone and furosemide were adminis- tered. The treatment enabled successful weaning from mechanical ventilation after 1 week. The patient’s 1-year follow-up with sildenafil administration at the same dose was uneventful. At the age of 15 months and weight of 10 kg, the girl was readmitted in order to perform heart catheterization with pulmonary artery (PA) pressure mea- surement. At that time diaphoresis during activity was noted in the anamnesis. In TTE 11 × 9 mm ASD with left- to-right shunt, two insignificant small residual muscular VSDs and right heart enlargement were observed. The PA pressure of 34/9/22 mm Hg and RV of 37/0/9 mm Hg were measured. Therefore, successful percutaneous ASD closure with a 12 mm Cocoon Septal Occluder (Vascular Innovations Co., Nonthaburi, Thailand, 8 Fr sheath) was performed under transesophageal guidance without bal- loon calibration (Figures 3, 4). In a 15-month follow-up the child was asymptomatic, TTE did not show residual leak through the ASD, and RV dimensions decreased, al- though on a decreasing dose of sildenafil.

Surgical closure of muscular VSDs in small infants is technically challenging [1]. Especially VSDs located api- cally are difficult to identify surgically. Hybrid VSD closure with Amplatzer occluders has become an attractive al- ternative option [2]. Recently published multicenter mid- term results have confirmed high efficacy and safety of such an approach and avoidance of cardiopulmonary by-

pass [2]. Our preliminary experience also indicates suit- ability of devices other than Amplatzer, such as Hyperion (China, VSD) or Cocoon (Thailand, ASD). Percutaneous access is another useful alternative, although limited by the patient’s weight. Secundum ASD percutaneous clo- sure is the method of choice even in small children [3].

Pulmonary hypertensive crisis is an important cause of morbidity and mortality in patients with pulmonary Figure 3. Transesophageal echocardiography with color Doppler. Atrial septal defect with left-to-right shunt

LA – left atrium, RA – right atrium.

Figure 4. Fluoroscopy. 7 mm Hyperion VSD Mus- cular (below) and 12 mm Cocoon Septal Occluders

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Michal Galeczka et al. Hybrid approach for muscular VSD and ASD

114 Advances in Interventional Cardiology 2018; 14, 1 (51)

arterial hypertension secondary to congenital heart dis- ease who require intervention [4]. Non-restrictive VSD accompanied by a  large ASD may cause early develop- ment of PH. Sequentially performed interventions can counteract this process. Early hybrid VSD closure has be- come an attractive method of treatment, but it carries a potential risk of pulmonary hypertensive crisis in the post-procedural period.

Conflict of interest

The authors declare no conflict of interest.

References

1. Fishberger SB, Bridges ND, Keane JF, et al. Intraoperative device closure of ventricular septal defects. Circulation 1993; 88: 205-9.

2. Gray RG, Menon SC, Johnson JT, et al. Acute and midterm results following perventricular device closure of muscular ventricular septal defects: a multicenter PICES investigation. Catheter Car- diovasc Interv 2017; 90: 281-9.

3. Knop M, Szkutnik M, Fiszer R, et al. Transcatheter closure of atrial septal defect in children up to 10 kg of body weight with Amplatzer device. Cardiol J 2014; 21: 279-83.

4. Brunner N, de Jesus Perez V, Richter A, et al. Perioperative phar- macological management of pulmonary hypertensive crisis during congenital heart surgery. Pulm Circ 2014; 4: 10-24.

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