Kulik Thomas J, McSweeney Julia E, Tella Joseph, Mullen Mary P
Division of Cardiac Critical Care, and the Pulmonary Hypertension Program, Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02115, USA.
Harvard Medical School, Boston, MA, USA.
Pediatr Cardiol. 2019 Apr;40(4):719-725. doi: 10.1007/s00246-019-02054-x. Epub 2019 Jan 23.
Reports of "treat and repair" of cardiac shunting lesions with inoperably high pulmonary vascular resistance (PVR) mostly concern pre-tricuspid defects; post-tricuspid lesions are different. We report our experience with pulmonary artery (PA) banding ± targeted pulmonary hypertension medications in five patients with a large VSD and inoperably high PVR, and review previous reports of PA banding with post-tricuspid defects. Three of our 5 patients had mean PAP > 50 mmHg after banding and no or only a transient fall in PVR. Two patients had mean PAP < 50 mmHg and lower PVR after banding; they had closure of their VSDs but have since had a progressive increase in PVR (follow-up after closure, 3.5 and 7.7 years). Previous reports have also documented difficulty in achieving sufficient band gradient. Of previously reported patients, only one became operable only after banding and targeted therapy, and was repaired; follow-up after repair was short-term (16 months). Our and previous experience demonstrate the difficulty in placing a PA band sufficiently tight to substantially reduce PA pressure. Reported attempts to "treat and repair" post-tricuspid defects are few and have met with limited success, and we found that PVR may increase significantly over time after repair. But more information is needed. Accurate interpretation of experience with "treat and repair" requires: careful characterization of the pulmonary circulation prior to "treating"; considering spontaneously reversible factors at pre-treatment catheterization before ascribing reduction in PVR to medical therapy; and long-term observation of PVR in patients who have had defect closure.
关于对肺血管阻力（PVR）高到无法手术的心脏分流病变进行“治疗和修复”的报告大多涉及三尖瓣前缺损；三尖瓣后病变则有所不同。我们报告了对5例大室间隔缺损（VSD）且肺血管阻力高到无法手术的患者进行肺动脉（PA）环扎术±针对性肺动脉高压药物治疗的经验，并回顾了先前关于三尖瓣后缺损进行PA环扎术的报告。我们的5例患者中有3例在环扎术后平均肺动脉压（PAP）>50 mmHg，且PVR无下降或仅短暂下降。2例患者在环扎术后平均PAP<50 mmHg且PVR降低；他们的VSD得以闭合，但此后PVR逐渐升高（闭合后随访3.5年和7.7年）。先前的报告也记录了难以实现足够的环扎压差。在先前报告的患者中，只有1例仅在环扎术和针对性治疗后才具备手术条件并接受了修复；修复后的随访是短期的（16个月）。我们和先前的经验都表明，很难将PA环扎得足够紧以大幅降低PA压力。关于“治疗和修复”三尖瓣后缺损的报告很少，且取得的成功有限，我们发现修复后PVR可能会随时间显著升高。但还需要更多信息。对“治疗和修复”经验的准确解读需要：在“治疗”前仔细描述肺循环情况；在将PVR降低归因于药物治疗之前，考虑治疗前导管检查时的自发可逆因素；以及对缺损已闭合患者的PVR进行长期观察。
