Beating-Heart Surgery for The beating-heart Norwood operation was performed

Hypoplastic Left Heart SyndromeWith Coronary Artery FistulasShunsuke Matsushima, MD,Yoshihiro Oshima, MD, PhD, Ayako Maruo, MD, PhD,Tomomi Hasegawa, MD, PhD,Hironori Matsuhisa, MD, PhD, Rei Noda, MD, andRyuma Iwaki, MD

Department of Cardiovascular Surgery, Kobe Children’sHospital, Kobe, Japan

The presence of ventriculocoronary connections in pa-tients with hypoplastic left heart syndrome and mitralstenosis-aortic atresia is a suggested risk factor formyocardial ischemia after surgical palliation. Wedescribe a neonate with this anatomic variant of hypo-plastic left heart syndrome who underwent a successfulbeating-heart Norwood operation, with continuous coro-nary perfusion. The ventricular condition could be visu-ally confirmed during the procedure, and a postoperativeechocardiogram showed preserved ventricular function.This technique is considered an effective option forminimizing myocardial damage in this patient subtype.

(Ann Thorac Surg 2014;98:e103–5)� 2014 by The Society of Thoracic Surgeons

entriculocoronary connections (VCCs), also called

Vventriculocoronary fistulas or sinusoids, are fre-quently seen in patients with the mitral stenosis-aorticatresia (MS-AA) subtype of hypoplastic left heart syn-drome (HLHS) [1, 2]. The presence of VCCs is a risk factorfor early failure after stage I palliation [1]. However, theexact mechanism for the increased mortality in this sub-type remains to be elucidated. Here, we describe aneonate with HLHS and extensive VCCs who underwenta successful, modified, beating-heart Norwood operation,with continuous coronary perfusion.

A 2.93-kg male neonate was born at 39 weeks gestation,with a prenatal diagnosis of HLHS and MS-AA, requiringprostaglandin E1 infusion. Transthoracic echocardiogra-phy confirmed the diagnosis and the presence of exten-sive VCCs (Fig 1). Color Doppler flow mapping showedprominent systolic antegrade flow through the VCCs anddiastolic retrograde flow through the 4.5-mm diameterascending aorta. Gentle balloon atrial septostomy wasperformed and subsequent bilateral pulmonary arterybanding was performed on postnatal day 3. In the im-mediate postoperative period transthoracic echocardiog-raphy showed transient ventricular dysfunction, owing tothe decreased left ventricular preload, that was resolvedby volume loading.

Accepted for publication June 24, 2014.

Address correspondence to Dr S. Matsushima, Department ofCardiovascular Surgery, Kobe Children’s Hospital, 1-1-1, Takakuradai,Suma-ku, Kobe, 654-0081, Japan; e-mail: matsushima_kch@hp.pref.hyogo.jp.

� 2014 by The Society of Thoracic SurgeonsPublished by Elsevier

on postnatal day 29, when the patient weighed 3.46 kg.After a median sternotomy, an arterial cannula wasinserted through a 3-mm expanded polytetrafluoro-ethylene tube (GoreTex; W.L. Gore & Assoc., Flagstaff,AZ) sewn onto the brachiocephalic artery. Bicaval venousdrainage was achieved and a cardiopulmonary bypass(CPB), with mild hypothermia (34�C), was initiated. Asecond arterial cannula was placed in the descendingaorta just above the diaphragm. After clamping thedescending aorta and ligating the aortic isthmus, theductal tissue was completely excised and the descendingaorta was transected proximally. Additional clamps wereplaced on the proximal aortic arch, left subclavian artery,and left common carotid artery prior to performing aorticarch reconstruction. The transected main pulmonary ar-tery was joined to the transected descending aorta with aglutaraldehyde-treated, autologous pericardial patchwhich augmented the lesser curvature of the newlyreconstructed aortic arch, and this amalgamation wasanastomosed to the longitudinally opened aortic arch.

Fig 1. Preoperative, color Doppler flow mapping echocardiograms.(A) Parasternal short-axis view showing extensive ventriculocoronaryconnections (white arrows). (B) Parasternal long-axis view showingprominent antegrade systolic flow in the ascending aorta (yellowarrow). (aAo ¼ ascending aorta; LA ¼ left atrium; LV ¼ left ventricle;RV ¼ right ventricle.)

0003-4975/$36.00http://dx.doi.org/10.1016/j.athoracsur.2014.06.112

Fig 2. Schematic diagram of the beating-heart Norwood procedure.Continuous coronary perfusion was maintained through an 18-gaugecardioplegia spike (CP spike). The lesser curvature of the newlyreconstructed aortic arch was augmented with a glutaraldehyde-treated, autologous pericardial patch (black arrow) joining the mainpulmonary artery (PA) to the descending aorta (dAo). Thisamalgamation was anastomosed to the longitudinally opened aorticarch. (aAo ¼ ascending aorta; BCA ¼ brachiocephalic artery;ePTFE ¼ expanded polytetrafluoroethylene; LCA ¼ left commoncarotid artery; LSCA ¼ left subclavian artery.)

e104 CASE REPORT MATSUSHIMA ET AL Ann Thorac SurgHIGH-RISK HLHS BEATING-HEART SURGERY 2014;98:e103–5

During the reconstruction of the aortic arch, rightventricle ischemic changes were observed. Then, an18-gauge cardioplegia spike was placed in the proximalascending aorta and connected to the side arm of thearterial circuit (Fig 2). Soon after the start of proximalcoronary perfusion complete recovery of right ventricularcontractions was confirmed and maintained during atrialseptectomy, arch reconstruction completion, and rightventricle to pulmonary artery (RV-PA) shunt placement,using a 6-mm GoreTex tube. Finally, the patient wasweaned from the CPB without any complications.

The patient’s postoperative course was uneventful.Repeated echocardiography revealed preserved rightventricular function and an unchanged pattern of coro-nary perfusion. A bidirectional Glenn shunt was per-formed when the patient was 5- months old. During theGlenn anastomosis, ventilation was continued, andthe left pulmonary blood flow was maintained throughthe RV-PA shunt to preserve left atrial oxygen saturation.After establishing the Glenn shunt, the RV-PA conduitwas subtotally excised. At the 6-month follow-up visit, thepatient’s condition was stable, with preserved ventricularfunction, and he was waiting for a Fontan procedure.

Comment

The presence of VCCs in patients with HLHS and MS-AAhas been implicated as a risk factor for increased hospital

mortality after surgical palliation. Vida and colleagues [1]found a 50% failure rate for stage I palliation in patientswith this anatomic variant and Sathanandam and col-leagues [2] suggested that large VCCs were associatedwith a poor prognosis. Although the exact mechanism forthe increased mortality in this disease subtype remainsunclear, some authors have proposed that the VCCsmight interfere with adequate myocardial protectionduring heart decompression on CPB, resulting inischemia-induced ventricular dysfunction [1, 3]. Thiscondition can be referred to as left ventricle-dependentcoronary circulation, as a similar condition involving theright side of the heart in patients with pulmonary atresiaand intact ventricular septum is called right ventricle-dependent coronary circulation. We hypothesized thatthe left ventricular systolic pressure might play a vital rolein creating coronary perfusion pressure, and that leftventricular collapse could be prevented by proximalcoronary perfusion in patients with extensive VCCs. Inthe present case, therefore, we employed the modifiedNorwood operation on a beating heart with continuouscoronary perfusion and ventricular contraction.Kishimoto and colleagues [4] first reported the beating-

heart Norwood operation, in which continuous coronaryperfusion was established using flow from the brachio-cephalic artery with an aortic cross-clamp at the level ofthe proximal arch [4]. Since then, several modificationshave been described [5, 6], including the use of contin-uous coronary flow using a cardioplesia spike or an olive-tip cannula secured in the proximal ascending aorta [6];the authors reported that this modification was a safealternative for use with any size of aorta. For smallerascending aortic diameters, an olive-tip cannula con-nected to the arterial circuit can be placed into the lumenof the opened aorta and secured with a vessel looptourniquet. In addition to their technique we employedmild hypothermic CPB, with lower body perfusion [7], tofurther maintain the ventricular contractions.Despite the use of this beating-heart strategy, a short

period of atrial decompression during atrial septectomy isunavoidable. In addition, the efficacy of this technique forpatients with the severest forms of VCCs, such as coro-nary artery interruption, remains unclear. However, themyocardial condition can be visually checked during theprocedure, which is another advantage of this beating-heart technique.In summary, the beating-heart Norwood operation,

with continuous coronary perfusion, is an effective optionfor providing myocardial protection in HLHS patientswith MS-AA and large VCCs.

References

1. Vida VL, Bacha EA, Larrazabal A, et al. Surgical outcome forpatients with the mitral stenosis–aortic atresia variant of hy-poplastic left heart syndrome. J Thorac Cardiovasc Surg2008;135:339–46.

2. Sathanandam S, Cui W, Nguyen NV, et al. Ventriculocoronaryartery connections with the hypoplastic left heart: a 4-yearprospective study: incidence, echocardiographic and clinicalfeatures. Pediatr Cardiol 2010;31:1176–85.

e105Ann Thorac Surg CASE REPORT MATSUSHIMA ET AL2014;98:e103–5 HIGH-RISK HLHS BEATING-HEART SURGERY

3. Polimenakos AC, Sathanandam SK, Husayni TS, El Zein CF,Roberson DA, Ilbawi MN. Hypoplastic left heart syndromeand aortic atresia-mitral stenosis variant: role of myocardialprotection strategy and impact of ventriculo-coronary con-nections after stage I palliation. Pediatr Cardiol 2011;32:929–39.

4. Kishimoto H, Kawahira Y, Kawata H, Miura T, Iwai S, Mori T.The modified Norwood palliation on a beating heart. J ThoracCardiovasc Surg 1999;118:1130–2.

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6. Turek JW, Hanfland RA, Davenport TL, et al. Norwoodreconstruction using continuous coronary perfusion: a safeand translatable technique. Ann Thorac Surg 2013;96:219–23.

7. Imoto Y, Kado H, Shiokawa Y, Fukae K, Yasui H. Norwoodprocedure without circulatory arrest. Ann Thorac Surg1999;68:559–61.