Combined Bentall and modified Ravitch procedures in a patient with Marfan syndrome
A 14-year-old boy, known case of Marfan syndrome, presented with chief complaints of dyspnea, while playing, which had progressed in last two months to dyspnea on routine activities. Patient had a typical Marfan habitus with arm span > height (194 > 179 cm), chest deformity (pectus excavatum; ), and kyphoscoliosis. He had a normal airway (Mallampatti class I). Past history included retinal surgery and lens replacement for retinal detachment and lens dislocation. Diagnosis of Marfan syndrome was made in accordance with the Ghent criteria.[] Cardiovascular examination revealed diastolic murmur at apex. Routine laboratory values were unremarkable. Chest X-ray showed scoliosis with convexity to left, crowding of ribs on right side, kyphosis in lower thoracic region, and marked displacement of the heart to the left. Pulmonary function tests revealed moderate restrictive ventilation defect. Transthoracic echocardiography showed tricuspid aortic valve with severe aortic regurgitation, no aortic stenosis, aortic annulus 26 mm, severely dilated aortic root (68 mm), aneurysmal ascending aorta extending up to origin of right brachiocephalic artery, no aortic dissection and normal ventricular function. Computed Tomography of chest revealed a dilated aortic root, a fusiform aneurysmal proximal ascending aorta, severe pectus excavatum (5.2 cm central depression of the sternum), displacement of the heart into left side of chest with compression of left lung and kyphoscoliosis []. Bentall procedure with concomitant chest wall repair was planned, and informed consent was taken for the same.\nGeneral anaesthesia was induced with intravenous (IV) midazolam (0.05 mg/kg), fentanyl (3 mg/kg), thiopentone (4 mg/kg) and pancuronium (0.1 mg/kg). Adequate care was taken to support the limbs during anesthesia and surgery. Intraoperatively standard cardiac monitoring was done including invasive arterial pressure, central venous pressure and transoesophageal echocardiography. Anesthesia was maintained with isoflurane in oxygen and intermittent doses of midazolam, fentanyl and pancuronium. Tidal volume of 7-8 ml/kg was used to maintain normocarbia. Surgery was initiated with midline incision and median sternotomy. Bone wax was used to control bleeding from sternal medulla. After systemic heparinization (400 IU/kg), aorto-bicaval cannulation was done. Aorta was cross clamped, heart was arrested with cold blood cardioplegia and patient cooled to 28°C. A 25 mm St Jude mechanical valved conduit (St Jude Medical, Inc, St Paul, MN.) was placed and coronary buttons were implanted to the conduit. Aortic cross clamp time was 99 minutes and cardiopulmonary bypass time was 144 minutes. After a smooth weaning from cardiopulmonary bypass, hemostasis was achieved.\nAfter achieving hemostasis, chest wall repair was started. The pectoral and rectus muscle flaps were elevated off the sternum and costal cartilage. Total subperichondrial resection of the deformed costal cartilages was achieved by incising the perichondrium anteriorly, and the entire third to eighth cartilages were removed to the costochondral junctions bilaterally. The sternum was dissected free from the intercostal neurovascular bundles bilaterally, medial to the internal mammary arteries. The deformed portion of the sternum was transacted in two points, and the divided sternum was fixed and corrected in elevated position by using a dynamic compression plate (3.5 mm/15 holes, Synthes Holding AG, Solothurn, Switzerland) passed horizontally posterior to the ribs to support the lower xiphisternum []. Following the correction of the deformity, all intercostal bundles were attached to the sternum. The plate was fixed to the xiphisternum and to the ribs bilaterally with steel wires. The pectoral and rectus muscles were reattached to the sternum after positioning of mediastinal and pleural drains. Routine closure of sternum was done with steel wires. Total duration of the surgery was six hours.\nPostoperative analgesia was achieved with IV morphine infusion (10-30 mg/kg/hr) till the patient was on mechanical ventilation, followed by IV paracetamol (15 mg/kg, six hourly). Patient was hemodynamically stable, and trachea was extubated on day one. Patient had a good cosmetic repair [] and was discharged from the hospital on tenth day.