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J Thorac Cardiovasc Surg 2007;133:1654-1656
© 2007 The American Association for Thoracic Surgery


Brief Communication

Hybrid procedure for the neonatal management of pulmonary atresia with intact ventricular septum

Hao Zhang, MD, PhDa, Shou-jun Li, MDa, Yong-qing Li, MDb, Hao Wang, MD, PhDb, Sheng-shou Hu, MDa,*

a Center for Pediatric Cardiac Surgery, Cardiovascular Institute and Fu Wai Hospital, Chinese Academy of Medical Science, Beijing, China
b Department of Echocardiography, Cardiovascular Institute and Fu Wai Hospital, Chinese Academy of Medical Science, Beijing, China.

Received for publication January 30, 2007; accepted for publication February 6, 2007.

* Address for reprints: Sheng-Shou Hu, MD, Department of Cardiac Surgery, Cardiovascular Institute and Fu Wai heart Hospital, Chinese Academy of Medical Sciences, 167A Beilishilu, Beijing 100037, P.R. China. (Email: shengshouhu{at}yahoo.com).


Figure 1
Drs Li, Hu, and Zhang (left to right)


Early decompression of the right ventricle (RV) is crucial for the neonatal management of pulmonary atresia with intact ventricular septum (PAIVS) without abnormalities of the coronary artery circulation. We developed a novel hybrid beating heart procedure for the management of PAIVS.

Clinical Summary

From March 2005 through January 2007, 4 neonates (3–4.5 kg) with PAIVS were treated with the hybrid procedure in the first week after birth. All newborns received preoperative prostaglandin E1 infusion. The Z value of the tricuspid valve diameter was –1.5, –2, –2, and 1.5 separately, which was measured by means of 2-dimensional echocardiography. All newborns had a patent infundibulum, and absence of the trabecular portion occurred in 1 case.

The heart was exposed with a midline sternotomy. A purse-string suture was placed in the right ventricular outflow tract (RVOT) 2 cm away from the pulmonary trunk. The operator held the echocardiographic probe in the subxiphoid to capture a clear image for the atretic pulmonary valve (PV), and then a 16-gauge intravenous catheter (BD Angiocath, Infusion Therapy Systems) was punctured perventricularly and perforated the PV. A guide wire was then inserted into the sheath and used to guide the balloon (Numed) across the PV (Figure 1). Sequential dilations were performed until a full opening of the PV with the guidance of epicardial echocardiography was obtained (Figure 2). A 3.5-mm modified Blalock–Taussig (BT) shunt was placed after successful balloon valvuloplasty. Finally, the prostaglandin infusion was ceased, and the patent ductus arteriosus was ligated.


Figure 1
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Figure 1. Scheme of the hybrid procedure. A, Puncture through the right ventricle free wall. B, Balloon valvuloplasty guided with epicardial echocardiography.

 

Figure 2
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Figure 2. Balloon valvuloplasty guided with epicardial echocardiography. A, The atretic pulmonary valve. B, Full opening of the pulmonary valve after repeated balloon dilation.

 
The newborns were ventilated for 6 to 72 hours after the operation. They were discharged with arterial oxygen saturation values of 75% to 88%. No additional procedures were needed, and both growth of the RV and body weight were regained during the follow-up period (1–23 months). Mild PV stenosis was observed in 1 child, and mild-to-moderate PV regurgitation was seen in all children. One child with a well-developed RV was scheduled for percutaneous occlusion for the BT shunt to achieve definitive repair, and another with relatively right ventricular hypoplasia was scheduled for a bidirectional Glenn procedure. The other 2 patients, with arterial oxygen saturation values of 87% to 91%, were still waiting for adequate growth of the RV.

Discussion

With the development of catheter-based devices, percutaneous catheter-based therapy has been advocated for neonates with a patent infundibulum and a lack of RV-dependent coronary circulation.1Go However, percutaneous perforation and balloon valvuloplasty were associated with higher rates of procedural failure and serious complications. Unfortunately, even after successful percutaneous balloon valvuloplasty, 43% to 51% of neonates needed urgent procedures, including BT shunt placements, RVOT reconstruction, or both.2,3Go Furthermore, in developing countries percutaneous laser– or radiofrequency-assisted perforation for neonates is far from popular and can be performed in very few centers. Hence, in general neonates with PAIVS were referred to the surgical department in our center. Surgical approaches, including RVOT reconstruction with a transannular patch and transventricular/pulmonary valvotomy, were also attempted to achieve right ventricular decompression; however, the mortality was considerably high.4Go Based on the above facts, we integrated the catheter-based devices and the traditional surgical procedure for PAIVS as initial management.

In this hybrid beating-heart procedure, first we perforate the PV with the needle and then deliver the balloon through the RV free wall to the pulmonary trunk. Epicardial echocardiography was used not only to guide the procedure with real-time imaging but also to evaluate the effectiveness of valvuloplasty with monitoring of the trans-PV flow and gradient pressure. In percutaneous catheter therapy, if the newborn could not be weaned from the prostaglandin infusion, an urgent BT shunt would be considered. Such a delayed decision could result in postoperative hypoxia and thus increased mortality. Because the operative field was offered by the open-chest approach, we regularly placed a BT shunt simultaneously. Ligation of the patent ductus arteriosus was performed to prevent the excess augmentation of blood flow to the lung, and then prostaglandin infusion was ceased immediately in the operating room.

We have not yet applied the hybrid procedure for PAIVS in a patient with a severely hypoplastic RV. Theoretically, such a hybrid procedure would augment the antegrade flow and offer the chance of development of the RV; therefore it was also beneficial for this subgroup.

In conclusion, our results show that, combined with real-time echocardiographic guidance and a catheter-based device, the hybrid procedure could be a good alternative to percutaneous catheter-based therapy for selected neonates with PAIVS, especially in centers in developing countries where the materials for percutaneous perforation are not available and the experience with neonatal percutaneous catheter therapy is limited.

Acknowledgments

We thank the National Eleventh-Five Year Project and Beijing Nova Project (2006 A85).

References

  1. Humpl T, Söderberg B, McCrindle BW, Nykanen DG, Freedom RM, Williams WG, et al. Percutaneous balloon valvotomy in pulmonary atresia with intact ventricular septum: impact on patient care. Circulation 2003;108:826-832.[Abstract/Free Full Text]
  2. Mclean KM, Pearl JM. Pulmonary atresia with intact ventricular septum: initial management. Ann Thorac Surg 2006;82:2214-2220.[Abstract/Free Full Text]
  3. Agnoletti G, Piechaud JF, Bonhoeffer P, Aggoun Y, Abdel-Massih T, Boudjemline Y, et al. Perforation of the atretic pulmonary valve: long-time follow up. J Am Coll Cardiol 2003;41:1399-1403.[Abstract/Free Full Text]
  4. Bull C, Kostelka M, Sorensen K, de Leval. Outcome measures for the neonatal management of pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 1994;107:359-366.[Abstract/Free Full Text]



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