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Fate of the “opened” arterial duct: Lessons learned from bilateral pulmonary artery banding for hypoplastic left heart syndrome under the continuous infusion of prostaglandin E1

2007; Elsevier BV; Volume: 133; Issue: 6 Linguagem: Inglês

10.1016/j.jtcvs.2007.01.053

1097-685X

Yoshihide Mitani, Shin Takabayashi, Hirofumi Sawada, Hiroyuki Ohashi, Hidetoshi Hayakawa, Yukiko Ikeyama, Kyoko Imanaka‐Yoshida, Kazuo Maruyama, Hideto Shimpo, Yoshihiro Komada,

Cardiovascular Conditions and Treatments

Recently, the Norwood stage I operation strategy for hypoplastic left heart syndrome (HLHS) has been challenged by proponents of the simultaneous Norwood/Glenn procedure after stage I bilateral pulmonary artery banding (PAB) using the ductal stenting technique.1Akintuerk H. Michel-Behnke I. Valeske K. Mueller M. Thul J. Schranz D. et al.Stenting of the arterial duct and banding of the pulmonary arteries: basis for combined Norwood stage I and II repair in hypoplastic left heart.Circulation. 2002; 105: 1099-1103Crossref PubMed Scopus (239) Google Scholar However, the latter strategy is invasive, necessitating a hybrid approach and inherent preductal aortic coarctation.2Bacha E.A. Daves S. Hardin J. Abdulla R.I. Anderson J. Hijazi Z.M. et al.Single-ventricle palliation for high-risk neonates: the emergence of an alternative hybrid stage I strategy.J Thorac Cardiovasc Surg. 2006; 131 (e2. Epub 2005 Dec 5): 163-171Abstract Full Text Full Text PDF PubMed Scopus (152) Google Scholar We recently reported a case of a patient with HLHS who successfully underwent the modified Fontan procedure after the Norwood/Glenn procedure after PAB, with the arterial duct kept open by administering lipo-prostaglandin E1 (lipo-PGE1) until the Norwood/Glenn procedure.3Takabayashi S. Shimpo H. Ozu Y. Yokoyama K. Kajimoto M. A Fontan completion through stage I bilateral pulmonary artery banding for hypoplastic left heart syndrome.J Thorac Cardiovasc Surg. 2005; 130: 1464-1465Abstract Full Text Full Text PDF PubMed Scopus (11) Google Scholar We report our novel observations in the natural history of PGE1-opened arterial ducts in our series. Five consecutive patients with HLHS underwent PAB in the neonatal period (10 ± 2 days), followed by the Norwood/Glenn procedure at 3 to 4 months of age (121 ± 10 days) after lipo-PGE1 infusion (3-5 ng/kg/min), on the basis of the first report (case 2)3Takabayashi S. Shimpo H. Ozu Y. Yokoyama K. Kajimoto M. A Fontan completion through stage I bilateral pulmonary artery banding for hypoplastic left heart syndrome.J Thorac Cardiovasc Surg. 2005; 130: 1464-1465Abstract Full Text Full Text PDF PubMed Scopus (11) Google Scholar in our series (Table 1). Serial angiograms, echocardiograms, and macroscopic and microscopic findings of the ductal tissues harvested in the operation were retrospectively investigated. During the interval between PAB and the Norwood/Glenn procedure, the ductal luminal patency was maintained in all 5 patients, as evaluated by echocardiography, angiography, and operative findings (Figure E1, Figure E2). However, localized intimal proliferation gradually developed at the aortic end of the duct in 4 patients (cases 2–5) before the Norwood/Glenn procedure, although such lesions were not obvious in 4 patients (cases 1–4) before PAB (Figure E1, Figure E2). The peak flow velocity at the site was minimal (1.0 and 1.1 m/s) in cases 1 and 2, respectively, with a minimal ridge in case 2 before the Norwood/Glenn procedure (Figure E1, b, e). A slight ridge (2.6 m/s) developed in case 3 with no pressure gradient, as determined by catheterization before the Norwood/Glenn procedure (Figure E1, h, i). A significant ridge (3.3 m/s) developed in case 4, with a pressure gradient of 30 mm Hg as determined by catheterization, at 107 days of age (Figure E2, b, c), although there was a slight ridge (2.3 m/s), with no pressure gradient as evaluated by catheterization, at 58 days of age. Case 5 had an obvious ridge (2.1 m/s) even before PAB (Figure E2, e) and underwent pulmonary artery–descending aorta grafting at 33 days of age because of the significant ductal stenosis (3.0 m/s). Ductal stenosis progressed to a pinhole lesion before the Norwood/Glenn procedure, whereas the ductal lumen was patent under lipo-PGE1 infusion (Figure E2, f, g). Macroscopic findings revealed the luminal patency of the ducts, of which circumferential ridges at the aortic sides were found in cases 4 and 5 (Figure E2, d, h). Microscopic findings showed the fibrocellular intimal lesion with the development of vaso vasorum in the ridges in cases 4 and 5 (Figure E2, i-k). The isthmus was widely open in all cases (5.7 ± 0.5 mm), without any signs of retrograde preductal aortic coarctation. No obvious systemic side effects of low doses of lipo-PGE1 were found. Cases 1, 2, and 4 survived the Norwood/Glenn procedure, among whom cases 1 and 2 completed the Fontan procedure successfully at 2 years of age, and case 4 awaits the Fontan procedure. Case 3, with associated chromosomal anomaly and intact atrial septum at the onset, and case 5, with associated multiple extracardiac anomalies, died after the Norwood/Glenn procedure because of pulmonary hypertension crisis and sudden circulatory collapse, respectively.TABLE 1Clinical characteristics of patientsCaseDiagnosisAnomalyBBW (kg)aAo diameter (mm)at PABat N/GAge (d)AgeBW (kg)Isthmus diameter (mm)1AA, MA3.52.0171244.75.22AS, MS3.45.17944.44.33AS, MS, IASChromosome anomaly3.24.471063.27.34AA, MAFacial anomaly3.12.781344.35.55AA, MSAnal atresia3.21.9121494.16.2VSDEar canal atresiaAA, Aortic atresia; AS, aortic stenosis; MA, mitral atresia; MS, mitral stenosis; IAS, intact atrial septum; VSD, ventricular septal defect; BBW, birth body weight; aAo, ascending aorta; PAB, pulmonary artery banding; BW, body weight; N/G, simultaneous Norwood Glenn procedure. Open table in a new tab Figure E2Echocardiograms, pulmonary angiograms, and macroscopic and microscopic findings of ductal tissues in cases 4 and 5. Echocardiograms delineating longitudinal sections of the arterial ducts obtained before PAB (a, e) and before the simultaneous Norwood/Glenn procedure (b, f), before which the lateral views of pulmonary angiograms (c, g) were obtained. Continuous Doppler flow velocity in echocardiograms (left lower corner) and pressure gradient across the duct in pulmonary angiograms (left lower corner). Macroscopic (d, h) and microscopic (i, j, k) photographs were obtained from the ductal tissue harvested in the operation. Luminal patency of the duct was maintained until the simultaneous Norwood/Glenn procedure (b, c, d, f, g, h). However, an echocardiographically obvious ridge with a pressure gradient of 30 mm Hg developed at the aortic end of the duct in case 4 (b, c, d). An echocardiographically obvious ridge with a continuous Doppler flow velocity of 2.1 m/s was found at the aortic end of the duct even before PAB in case 5 (e). The ridge developed to a pinhole-like lesion before the simultaneous Norwood/Glenn procedure (f, g, h). Fibrocellular intimal cushion formation (i, k) with the development of vaso vasorum (j, k) was found in the macroscopic ridges (d, h). Arterial ducts (yellow arrows); ridges (yellow asterisk).View Large Image Figure ViewerDownload Hi-res image Download (PPT) AA, Aortic atresia; AS, aortic stenosis; MA, mitral atresia; MS, mitral stenosis; IAS, intact atrial septum; VSD, ventricular septal defect; BBW, birth body weight; aAo, ascending aorta; PAB, pulmonary artery banding; BW, body weight; N/G, simultaneous Norwood Glenn procedure. The major findings in our series are two-fold: PGE1 infusion in HLHS after PAB produced an unexpectedly long duration of ductal luminal patency without preductal coarctation; however, the intimal proliferation gradually developed at the aortic end of the duct in the absence of functional closure, which unexpectedly resulted in hemodynamically significant stenosis of the duct in limited cases. Long-term ductal luminal patency during PGE1 infusion in HLHS after PAB, mimicking the environment of the duct in utero, is consistent with the increased incidence of patent ductus arteriosus in neonates with pulmonary hypertension and exaggerated vasodilator responses of the duct to PGE2 in neonatal lambs with bilateral PAB.4Clyman R.I. Mauray F. Heymann M.A. Roman C. Influence of increased pulmonary vascular pressures on the closure of the ductus arteriosus in newborn lambs.Pediatr Res. 1989; 25: 136-142Crossref PubMed Scopus (25) Google Scholar These findings confer the opportunity for the later second-stage Norwood operation, even with the Glenn procedure. The localized intimal proliferation in this situation is consistent with the developmentally regulated matrix remodeling, which was previously correlated to the postnatal anatomic closure of the duct in newborn lambs.5Rabinovitch M. Cell-extracellular matrix interactions in the ductus arteriosus and perinatal pulmonary circulation.Semin Perinatol. 1996; 20: 531-541Abstract Full Text PDF PubMed Scopus (38) Google Scholar These findings may be a potential hazard for the long-term ductal opening in limited cases. Collectively, the present findings may give us a biological basis and a caution for our minimally invasive strategy against HLHS, as well as a fundamental insight into the biology of the "PGE1-opened" arterial duct in humans. Further studies are warranted in this regard.