Evaluation of pressures before intervention in the operation room and after correction are illustrated in the (Table
1.). Immediately after completion of surgery (STS), the pressure of PVC decreased to the point where its pressure gradient became zero. Blood oxygenation improved up to 84% (preoperative Sao2 was 70% on 100% oxygen) and cyanosis, agitation, feeding problem subsided. Three days later, when he was discharged, arterial oxygen saturation had reached as high as 91%. Despite good advances in treating of TAPVC in recent decades, this severe malformation in its various anatomical forms remains a challengeable entity during early infancy. Significant obstruction to pulmonary venous drainage results in pulmonary edema in the presence of a normal size and shape of the heart and cardiogenic shock which is rapidly lethal if untreated. Our patient preoperatively suffered from significant gradient between the drainage point of vertical vein to the left brachiocephalic vein and the pulmonary veins with flow acceleration > 3.0 m/sec (pulmonary venous obstruction) but in postoperative echocardiography, midflow acceleration 0.8 m/sec was found at the conduit site. This flow and patency was maintained up to the second operation, showing an identical median Vmax of 0.8 m/sec. Neither turbulent nor continious flow was observed in the conduit. Almost all reports have declared that perioperative high mortality associates with PVO, low weight (W < 2.5–3 kg), early age (A < 2 m), severe preoperative acidosis, long time of aortic Cross Clamp (ACC) and cardiac arrest. The second frontier in the treatment of TAPVC is represented by postoperative PVO. In such a difficult situations, if patients survive from operation, most of them will require multiple postoperative surgical interventions due to recurrent PVO with an increasingly poor outcome at each representation [
7]. Medical efforts are minimally effective in managing the ensuing hemodynamic and metabolic problems so their use is limited to provide some short lived conservative therapy until definitive surgical treatment is carried out. PVO is usually lethal, even with reoperation and extensive attempts at revision or repair [
8]. This lack of success has led to alternative treatments such as balloon dilatation and stenting. The Rashkind Operation or Balloon Atrial Septostomy (BAS) has been used with some success to decompress the pulmonary venous pressure and improve C/O in the restricted ASD, but these don’t appear to provide additional benefit. Moreover several reportshave proposed the use of percutaneous angioplasty and stenting of the obstructed vein to palliate shock and improve preoperative metabolic state. Research showed during the median cross - sectional follow up of 3.1 years, estimated mortality was 38+/− 8% at 1 year and 50+/− 8% at 5-years after stent implantation. Necessity for reintervention (owing to occlusion of stent), was 58+/− 7% at 1-year. In 1996 sutureless repair technique was described, using in situ autologous pericardium for recurrent pulmonary vein stenosis following main TAPVC surgery [
9]. Subsequent reports emphasize the utility of this technique in selected patients as main procedure. Despite interest in the sutureless technique, there is little firm evidence that it provides a benefit over conventional techniques used a retrospective analysis to compare the outcomes of death and restenosis after conventional and sutureless techniques. By multivariable analysis, there was no statistically significant difference between the conventional and sutureless techniques. We encountered with a patient, who had almost encompassed all critical risk factors that were sufficient to make the operative prognosis very poor. Routine conventional operative procedures have reported mortality rate up to %50 and post operative pulmonary venous obstruction (PVO) morbidity rate up to %54 especially in the obstructive type of TOPVC as well as almost all of them need to the secondoperation. Our patient took more opportunity to gain appropriate weight also heart chambers and pulmonary veins grew adequately. The Sarmast - Takriti Shunt (STS) stablished adequate postoperative hemodynamics for symptomatic neonate and prompt left cardiac side rehabilitation. The STS with confined heparin (100 U/kg), was carried out without using CPB with an intention to reduce the morbidity associated with extra corporeal circulation. Eliminating CPB reduced the cost of the procedure substantially and saved the patient from its inherent complications [
10]. .After procedure the enough time was prepared on behalf of the heart to compensate its chambers especially the right ventricle and left atrium and ensure endurable state for the main surgery. Although our experience was limited to STS in supracardiac type, we are optimistic and hopeful to its feasibility and usefulness in other types of TAPVC. Now, we are so satisfied owing to be able to help such a complicated neonate.
Table 1
Preoperation and postoperative cardiac pressures of 4-days old male with total anomalous pulmonary venous connection accompanied by pulmonary venous obstruction
Preoperative pressures (mmHg) | 29 | 9 | 8 | 9 | 61/13 | 59/31 |
Postoperative pressures (mmHg) | 8 | 8 | 9 | 8 | 32/10 | 28/15 |