Khan Inam Ullah, Ahmed Iftikhar, Mufti Waqar A, Rashid Azhar, Khan Asif Ali, Ahmed Syed Afzal, Imran Muhammad
Department of Cardiac Surgery, Armed Forces Institute of Cardiology & National Institute of Heart Diseases (AFIC/NIHD), Rawalpindi, Pakistan.
J Ayub Med Coll Abbottabad. 2006 Oct-Dec;18(4):21-5.
To evaluate the surgical and medical efficacy of the patients operated for Ventricular Septal Defect (VSD) with Pulmonary Hypertension and Pulmonary Vascular Resistance (PVR). Infants and children with elevated PVR and Pulmonary Hypertension are associated with significant mortality and morbidity after surgical closure. Circulatory assist devices and sophisticated medicines may not be available to help in the management of infants and children with elevated Pulmonary artery pressure and resistance. We left Patent Foramen Ovale (PFO) or made atrial communication to decrease the morbidity and mortality associated with the closure of large VSD in this risky group.
Sixteen infants and children were operated with median age of 12 months, operated by the same surgeon (IU), from January' 2004 to December' 2005. They were with large VSD of elevated PVR (3.9+0.3) and underwent VSD closure leaving PFO or artificial ASD (5mm). Surgical approach was through right atrium. Post operatively, all the patients were electively ventilated for 36 hours. They were given intravenous dilators (Glyceral Trinitrate + Phentolamine) and oral Sildenafil up to 1 mg /Kg, six hourly. Five cases went into acute pulmonary hypertensive crisis postoperatively, and were rescued by Prostacycline Nebulization.
Sixteen patients had VSD as the primary lesion that underwent operation. The overall early mortality was 6.25% (1/16). There have been no late deaths.
Closure of large VSD with elevated PVR can be performed, leaving PFO or artificial ASD, with acceptable mortality and morbidity.
评估患有肺动脉高压和肺血管阻力（PVR）的室间隔缺损（VSD）患者的手术及内科治疗效果。PVR升高和患有肺动脉高压的婴幼儿在手术闭合后具有显著的死亡率和发病率。循环辅助装置和复杂的药物可能无法用于帮助管理肺动脉压力和阻力升高的婴幼儿。我们保留卵圆孔未闭（PFO）或制造心房交通，以降低这一高危组中大型VSD闭合相关的发病率和死亡率。
2004年1月至2005年12月，16例婴幼儿接受手术，中位年龄12个月，由同一位外科医生（IU）实施手术。他们患有PVR升高的大型VSD（3.9 + 0.3），并接受了VSD闭合术，保留PFO或人工房间隔缺损（5mm）。手术入路为右心房。术后，所有患者均接受选择性通气36小时。给予静脉扩张剂（硝酸甘油 + 酚妥拉明）和口服西地那非，剂量达1mg / Kg，每6小时一次。5例患者术后发生急性肺动脉高压危象，通过前列环素雾化吸入进行抢救。
16例患者以VSD作为主要病变接受手术。总体早期死亡率为6.25%（1/16）。无晚期死亡病例。
对于PVR升高的大型VSD，可以在保留PFO或人工ASD的情况下进行闭合，死亡率和发病率可接受。
