Background: Cyanotic congenital heart diseases present early in life with poor general condition of the patient. Majority of deaths occurs within one year of life before surgical intervention due to severe cyanosis and metabolic acidosis. Modified Blalock-Taussig Shunt (MBTS) is one of the palliative cardiac surgeries done for cyanotic congenital heart diseases. It improves the general condition of the patient before definitive surgery is done. The aim of this study was to determine the commonest indications, post-operative anticoagulation and early complications following MBTS at CARE Hospital, India.Methods: This was a retrospective study from January 2004 to December 2006 including all patients who underwent Posterolateral Thoracotomy for MBTS. All patients had deep cyanosis, oxygen saturation of 65% or less and small pulmonary vasculature due to congenital heart defects. Acyanotic patients and those with oxygen saturation more than 65% were excluded from the study. All patients received a single dose of heparin intra-operatively and oral aspirin as anticoagulant regimen post-operatively. No heparin given postoperatively.Results: A total of 20 children with a mean age of 27.4 months were studied. Two patients had pre-operative ICU admission due to severe cyanosis (both had oxygen saturation of 35%), hypotension and severe body weakness. The commonest indications for MBTS included Tetralogy of Fallot (70%), pulmonary atresia (10%) with or without Ventricular Septal Defect (VSD), tricuspid atresia (10%) with pulmonary atresia or stenosis and Double Outlet Right Ventricle (DOVR) with pulmonary atresia or stenosis (10%). Mean duration of ICU stay was 2 days, mean duration of mechanical ventilation was four and half hours, mean duration of hospital stay was 7 days and mean systemic oxygen saturation improved significantly from 46% to 84% ( x2 = 7.03, p = 0.0080). No post-operative bleeding, seroma, shunt thrombosis or death occurred in this study.Conclusion: The commonest indication for MBTS is TOF. Intra-operative single dose of heparin followed by post-operative oral aspirin as anticoagulant regimen was not associated with a major complication in terms of bleeding, seroma, shunt thrombosis, or death

Mannam, GC

Nyawawa, ETM

Pathuri, SN

Raju, DSB

Saikiran, KVSS

Sajja, LR

Sompalli, S

Ussiri, EV

English

Mpoki, U

Lugazia, ER

Sompali, S

AJOL - African Journals Online

23  East and Central African Journal of Surgery Volume 12 Number 2      November /December 2007  Modified Blalock-Taussig Shunt in Palliative Cardiac Surgery  E.V. Ussiri1, E.T.M. Nyawawa1, U. Mpoki2, E.R. Lugazia2, G.C. Mannam3, L.R. Sajja4.  S. Sompali4       1Specialist Surgeon, Cardiothoracic Surgery,  2Cardiac Anaesthesiology & Critical Care, 3Chief Cardiothoracic Surgeon, 4Consultant Cardiothoracic Surgeon - CARE Hospital, Hyderabad-India. Correspondence to: Dr.Elijah V. Ussiri, E-mail: eussiri@yahoo.com Background: Cyanotic congenital heart diseases present early in life with poor general condition of the patient. Majority of deaths occurs within one year of life before surgical intervention due to severe cyanosis and metabolic acidosis. Modified Blalock-Taussig Shunt (MBTS) is one of the palliative cardiac surgeries done for cyanotic congenital heart diseases. It improves the general condition of the patient before definitive surgery is done. The aim of this study was to determine the commonest indications, post-operative anticoagulation and early complications following MBTS at CARE Hospital, India.                   Methods: This was a retrospective study from January 2004 to December 2006 including all patients who underwent Posterolateral Thoracotomy for MBTS. All patients had deep cyanosis, oxygen saturation of 65% or less and small pulmonary vasculature due to congenital heart defects. Acyanotic patients and those with oxygen saturation more than 65% were excluded from the study. All patients received a single dose of heparin intra-operatively and oral aspirin as anticoagulant regimen post-operatively. No heparin given post-operatively.                                                                                                                                Results: A total of 20 children with a mean age of 27.4 months were studied. Two patients had pre-operative ICU admission due to severe cyanosis (both had oxygen saturation of 35%), hypotension and severe body weakness. The commonest indications for MBTS included Tetralogy of Fallot (70%), pulmonary atresia (10%) with or without Ventricular Septal Defect (VSD), tricuspid atresia (10%) with pulmonary atresia or stenosis and Double Outlet Right Ventricle (DOVR) with pulmonary atresia or stenosis (10%). Mean duration of ICU stay was 2 days, mean duration of mechanical ventilation was four and half hours, mean duration of hospital stay was 7 days and mean systemic oxygen saturation improved significantly from 46% to 84% ( x2 = 7.03, p = 0.0080). No post-operative bleeding, seroma, shunt thrombosis or death occurred in this study.                    Conclusion: The commonest indication for MBTS is TOF. Intra-operative single dose of heparin followed by post-operative oral aspirin as anticoagulant regimen was not associated with a major complication in terms of bleeding, seroma, shunt thrombosis, or death. Introduction  Modified Blalock-Taussig Shunt is a common palliative surgery done for cyanotic congenital heart diseases associated with reduced pulmonary blood flow prior to definitive surgery1,2. It is one of the systemic to pulmonary artery shunts whereby a synthetic conduit mainly Polytetrafluoroethylene - PTFE (Gore Tex) is anastomosed mainly between  right subclavian artery and right pulmonary artery or left side occasionally. Cyanotic children present with poor nutritional status, desaturation, metabolic acidosis and low cardiac output3,4. Haemodynamic and biochemical unstable patients require ICU admission for resuscitation and haemodynamic stabilization prior to MBTS5. The issue of post-operative heparin as anticoagulant to prevent shunt thrombosis has remained controversial as it differs from one center to another6,7. 24  East and Central African Journal of Surgery Volume 12 Number 2      November /December 2007  MBTS significantly improve growth of main, left and right pulmonary arteries and pulmonary valve annulus due to improved pulmonary blood flow4.  Consequently, these improve the general condition of the patient with good haemodynamic and biochemical status prior to definitive correction4,5. The commonest complications following MBTS include shunt thrombosis leading to obstruction, shunt stenosis, infection, peri-shunt (graft) seroma, pseudoaneurysm and pulmonary artery distortion2. This retrospective study was aimed at determining the indications, post-operative anticoagulation and immediate complications following MBTS at Care Hospital, Hyderabad-India.  Patients and Methods A retrospective study was undertaken from January2004 to December 2006. The study population included all patients who had Modified Blalock-Taussig Shunt (MBTS) at Care Hospital, India. The Theatre Register and Medical Records were used to obtain the necessary information from the patients’ files till the day of discharge from the hospital.  MBTS was indicated for all patients with deep cyanosis, oxygen saturation of 65% or less and small pulmonary vasculature by 2D echocardiography. Acyanotic patients and those with oxygen saturation above 65% were planned for definitive (primary) correction and therefore excluded from this study. Two patients had pre-operative ICU admission due to severe cyanosis (both had oxygen saturation of 35%) and hypotension. They were stabilized by ventilation, hydration, cardiac support with inotropic and ductal dilatation with Prostaglandin E1 infusion (0.05 µg/kg/min). Surgical approach was through conventional postero-lateral thoracotomy. All patients received heparin, 1 mg/kg intravenously stat just before shunt anastomosis. The size (diameter) of the shunt depends on the weight of the patient such that, neonate less than 2.0 kg = 3.0 mm diameter, infancy between 2.0 - 3.5 kg = 3.5 mm diameter and infancy above 3.5 kg = 4.0 mm diameter as well as intra-operative vascular assessment. Oral aspirin, 5mg/kg/day were started immediately on the arrival to ICU. No post-operative heparin given. Control Chest X-Ray and control 2D-echocardiography were done while in the ICU to exclude haematoma, to determine shunt patency, pulmonary artery anatomy and left ventricular function.   Results During this study, a total of 20 patients studied. Male to female ratio was 1.9:1 and 55% of all patients belong to infancy age group (Table 1). Two patients had pre-operative ICU admission for stabilization prior to MBTS. The commonest indication for MBTS was Tetralogy of Fallot which accounted for 70% of cases. Other indications were pulmonary atresia with or without VSD, tricuspid atresia with pulmonary atresia or stenosis and DORV with pulmonary atresia or stenosis (Table 2). Right MBTS accounted for 95% versus 5% for left MBTS. Mean duration for ICU stay was 2 days, mean duration for mechanical ventilation was four and half hours, mean hospital stay was 7 days, mean pre-operative oxygen saturation was 46% (Std Dev.= 7.20 ) and mean post-operative oxygen saturation was 84% (Std Dev.= 6.20 ){Table 3}. No post-operative bleeding, shunt thrombosis, peri-shunt seroma or death occurred in this study. Table1. To determine the age group per sex  Age(months)/sex Male Female Total  % 0-12 9 2 11  55 13-60 3 5 8  40 > 61 1 0 1  5 25  East and Central African Journal of Surgery Volume 12 Number 2      November /December 2007  Total 13 7 20  100 Male: Female = 1.9:1 Mean age = 27.4 (SD=28.91)   Table2.  To determine the Indications for MBTS   Table3. To determine the surgical outcome of MBTS Surgical outcome Number Mean duration of ICU stay(days) 2  (Std= 0.68) Mean duration of mechanical ventilation (hours) 4.30 (Std= 3.27) Mean duration of Hospital stay (days) 7 (Std=4.88) Mean preoperative Oxygen saturation (%) 46 (Std=7.20) Mean postoperative Oxygen saturation (%) 84 (Std=6.20) Bleeding 0 Shunt thrombosis 0 Peri-shunt seroma 0 Re-operation 0  Discussion A total of 20 children underwent MBTS. The Male: Female ratio was 1.9:1. Their mean age on presentation was 27.4 months. Infants above one month of age accounted for about 55% of all children. Two patients had pre-operative ICU admission for haemodynamic and biochemical stabilization prior to MBTS. Study by Sivakumar et al5 revealed 10 out of 20 neonates who underwent MBTS had pre-operative ICU admission for stabilization prior to MBTS. Gladman et al8 performed a MBTS in 65 children with a mean age of 58 days and 95% were done on the right side. Majority of patients in this study, 95% underwent right MBTS and only 5% done on the left side. Right MBTS make shunt ligation during definitive surgery easier compared to the left side. Left MBTS is mainly indicated for small left pulmonary artery anatomy compared to the right side. The commonest indications for MBTS in this study included Tetralogy of Fallot (70%), pulmonary atresia with or without VSD (10%), tricuspid atresia with pulmonary atresia or stenosis (10%) and DORV with pulmonary atresia or stenosis (10%). Similar findings were obtained by other studies with a single ventricle physiology anomaly associated with pulmonary atresia in addition 5,9. In this study no patient received post-operative heparin as an anticoagulant to prevent shunt thrombosis except aspirin. No shunt failure in terms of shunt thrombosis, peri-shunt (graft) seroma or bleeding. No patient who underwent thoracotomy for re-Diagnosis Number Tetralogy of Fallot 14 (70%) Pulmonary atresia with/without VSD 2 (10%) Tricuspid atresia + Pulmonary atresia/stenosis 2 (10%) DORV + Pulmonary atresia/stenosis 2 (10%) Total 20(100%) 26  East and Central African Journal of Surgery Volume 12 Number 2      November /December 2007  exploration and no death occurred in this study. The role of heparin in MBTS has remained controversial. Berger et al (6) found heparin as an independent risk factor for peri-graft seroma following MBTS but other studies concluded that heparin has no role post-operatively. Mullen et al (7) studied on 23 patients who underwent MBTS using a single dose of heparin intra-operatively and no post-operative heparin given. The findings revealed no post-operative bleeding or seroma developed, no shunt failure occurred during a mean follow-up of 18 months. It was concluded that shunt thrombosis is more likely related to intra-operative technical difficulty or extremely small pulmonary artery size. Gladman et al (8) found pulmonary artery distortion in about 33% of children who underwent MBTS and shunt stenosis was common and correlated with a younger age at palliation. MBTS improve the general condition of the patient. Our study revealed a remarkable improvement in terms of oxygen saturation, duration of mechanical ventilation, ICU and hospital stay and mortality. In this study, mean oxygen saturation was significantly improved from 46% to 84% (x2 =7.03, p = 0.0080), mean ICU stay was 2 days, mean duration of mechanical ventilation was 4 hours and 30 minutes, and mean hospital stay was 7 days post-operatively. Other studies had mean oxygen saturation improvement from 66% to 77% (4, 5), mean ICU stay of 5 days (5), peri-graft seroma less than 3% (2, 6, 8), shunt patency of 80% to 89% (5, 10) and mortality from 0 to 15% (4, 5, 10).  Conclusion   The commonest indication for MBTS is TOF. Intra-operative single dose of heparin followed by post-operative oral aspirin as anticoagulant regimen was not associated with a major complication in terms of bleeding, seroma, shunt thrombosis or death.  References 1. Sivakumar K, Anil SR, Ravichandra M, Natarajan KU, Kamath P, Krishna K. Emergency Transcatheter Balloon Re-canalization of Acutely Thrombosed Modified Blalock-Taussig Shunts. Indian Heart J. 2001; 53: 743- 748 2. Demircin M, Doon R, Ozkan M, Ozysoy F, Kuzgun E, Guvener M. Perigraft seroma complicating the MBTS: two consecutive instances with review of literature. Turkish JPaed 2004; 46: 275 – 278 3. Allan LD, Sharland GK. The echocardiographic diagnosis of totally anomalous pulmonary venous connection in the fetus. Heart 2001; 85: 433 - 437. 4. Kulkarni H, Rajani R, Dalvi B, Gupta KG, Vora  A, Kelkar P. Effect of Blalock-Taussig shunt on clinical parameters, left ventricular function and pulmonary arteries. J Postgrad Med 1995; 41: 34 -36  5. Sivakumar K, Shivaprakasha K, Suresh GR, Kumar RK. Operative Outcome and immediate term follow-up of neonate Blalock –Taussig shunts. Indian Heart J 2001; 53: 66 -70 6. Berger RM, Bol-Raap G, Hop WJ, Borgers AJ, Hess J. Heparin as a risk factor for perigraft seroma complicating the MBTS. J Thorac. Cardiovasc Surg199; 116: 286 – 293 7. Mullen JC, Lemermer G, Bentley MJ. Modified Blalock –Taussig shunts: To heparinized or not to heparinized. Cardiovasc Surg 1996; 12: 645 -647 8. Gladman G, McCrindle BW, Williams WG, Freedom RM, Benson LN. The Modified Blalock- Taussig Shunt: Clinical impact and morbidity in Fallot’s Tetralogy in thecurrent erra. J Thorac Cardiovasc Surg 1997; 114: 25 -30 9. Sahoo M, Salm M, Kale S, Saxena N. Serous fluid leakage following MBTS 27  East and Central African Journal of Surgery Volume 12 Number 2      November /December 2007  operation using PTEF grafts. Indian Heart J 2001; 53: 328 -331 10. Karpawich PP, Bush CP, Antilon JR, Amato JJ, Marbey ML, Agarwal KC. MBTSin infants and young children. Clinical and catheterization assessment. J Thorac Cardiovasc Surg 1985; 89: 275- 279  

Modified blalock-taussig shunt in palliative cardiac surgery

Background: Anomalous pulmonary venous connection (APVC) is a rare, life threatening congenital heart disease presenting early in life. It may be partial (PAPVC) or total (TAPVC). PAPVC mainly involve the right pulmonary arteries and is associated with sinus venosus atrial septal defect with a low morbidity and mortality. TAPVC is classified into supracardiac, intracardiac, infracardiac and mixed types. It carries a high morbidity and mortality. The aim of this study was to determine the morphological patterns and surgical outcome of APVC at CARE hospital, Hyderabad-India.Methods: This was a prospective study form January 2004 to December 2006. Study included all patients who underwent surgery for anomalous pulmonary venous connection and followed up to the time of discharge.Results: A total of 65 patients with APVC were studied where the results revealed 60% had TAPVC and 40% had PAPVC. Mean age of presentation was 21.6 months for TAPVC compared to 135.2 months for PAPVC. Among all patients with PAPVC 84.6 % had sinus venosus atrial septal defect. Supra-cardiac and intra-cardiac anomalous were the commonest type of TAPVC representing 43.6% and 35.9% respectively. Among all patients with TAPVC 51.35% were associated with ostium secundum atrial septal defect, 74.4% had moderate to severe pulmonary hypertension. Overall mortality was 9.25%. Mortality within TAPVC group was 15.4% and none from PAPVC.Conclusion: It was concluded that TAPVC present early in life and is associated with ostium secundum atrial septal defect, pulmonary hypertension, high morbidity and mortality. Accurate diagnosis and early correction improves the surgical outcome

18  East and Central African Journal of Surgery Volume 12 Number 2      November /December 2007  Patterns of Anomalous pulmonary Venous Connection as Seen at Care Hospital,  Hyderabad - India.  EV Ussiri1,2, ETM Nyawawa1, GC Mannam2, LR Sajja2 , DS Bhaskara Raju2, SN Pathuri2, KVSS Saikiran2, S Sompalli2.  1Muhimbili National Hospital, Tanzania, 2CARE Hospital, Hyderabad-India.  Correspondence to: Dr. EV Ussiri, CARE Hospital, Banjara Hills Road No.1, Hyderabad 500 034 India. E-mail address: eussiri@yahoo.com Background: Anomalous pulmonary venous connection (APVC) is a rare, life threatening congenital heart disease presenting early in life. It may be partial (PAPVC) or total (TAPVC).  PAPVC mainly involve the right pulmonary arteries and is associated with sinus venosus atrial septal defect with a low morbidity and mortality. TAPVC is classified into supracardiac, intracardiac, infracardiac and mixed types. It carries a high morbidity and mortality. The aim of this study was to determine the morphological patterns and surgical outcome of APVC at CARE hospital, Hyderabad-India. Methods: This was a prospective study form January 2004 to December 2006. Study included all patients who underwent surgery for anomalous pulmonary venous connection and followed up to the time of discharge. Results: A total of 65 patients with APVC were studied where the results revealed 60% had TAPVC and 40% had PAPVC. Mean age of presentation was 21.6 months for TAPVC compared to 135.2 months for PAPVC. Among all patients with PAPVC 84.6 % had sinus venosus atrial septal defect.  Supra-cardiac and intra-cardiac anomalous were the commonest type of TAPVC representing 43.6% and 35.9% respectively. Among all patients with TAPVC 51.35% were associated with ostium secundum atrial septal defect, 74.4% had moderate to severe pulmonary hypertension. Overall mortality was 9.25%. Mortality within TAPVC group was 15.4% and none from PAPVC.  Conclusion: It was concluded that TAPVC present early in life and is associated with ostium secundum atrial septal defect, pulmonary hypertension, high morbidity and mortality. Accurate diagnosis and early correction improves the surgical outcome.   Introduction  Anomalous Pulmonary Venous Connection (APVC) is one of the rare congenital heart diseases whereby, some or all of the pulmonary veins drain to the site other than left atrium1,2,3. Partial anomalous pulmonary venous connection (PAPVC) commonly involves the right pulmonary veins in more than 80% with normal drainage to the left side. About 90% of PAPVC are associated with Sinus Venosus Atrial Septal Defects -SVASD1,2,3.   Total anomalous pulmonary venous connection (TAPVC) involves all four pulmonary veins which form a common pulmonary chamber and drain into different    sites. It is classified according to the site of drainage as Supracardiac, intracardiac, infracardiac and mixed type as a combination of the mentioned types4,5. Definite diagnosis   of APVC is made by Trans-Thoracic Echocardiography and Trans-Esophageal Echocardiography (TEE) and rarely, cardiac catheterization is used when associated with other cardiac anomalies or  patient with age above 40 years to exclude coronary artery disease6,7,8. Clinical presentation and pathophysiology for PAPVC is similar to that of atrial septal defect (ASD) and the surgical treatment is directed towards closure of ASD while re-routing pulmonary veins into the left atrium using a patch. Surgical treatment carries a low morbidity and 0.9 % mortality rate3. TAPVC patients present with 19  East and Central African Journal of Surgery Volume 12 Number 2      November /December 2007  decompensated heart with desaturation and therefore, early presentation 9. Surgical treatment depends on the type of connection and this carries a high morbidity and mortality4,10,11. A prospective study was done to determine the morphological pattern and operative outcome of the APVC at CARE Hospital, Hyderabad- India.  Patients and Methods  A prospective study was done from January 2004 to December 2006 including all patients who underwent cardiac surgery for anomalous pulmonary connection (drainage).  Those patients whom their data could not be retrieved were excluded from the study. Operating theatre registry and medical record department were used to obtain the necessary details which included age, sex, type of anomaly and type of surgery done, number of veins and type of ASD involved in PAPVC, type of TAPVC and its associated anomalies, duration of mechanical ventilation. Also presence of pulmonary hypertension by echocardiography, pulmonary vein(s) obstruction and mortality were recorded. Pre-operative and post-operative echocardiography was done for diagnosis and control respectively. All patients were followed up to the time of discharge for any immediate complication.   Surgical technique  All patients with partial anomalies had glutaraldehyde treated pericardial patch closure of the ASD while re-routing pulmonary vein(s) into left atrium. Left pulmonary vein draining into innominate vein was divided and anastomozed to the left atrial appendage.  All patients with supra-cardiac and infra-cardiac total anomalies had posterior approach whereby the common pulmonary venous chamber was widely (more than 4 cm) anastomozed to the left atrium to prevent obstruction. All patients with intra-cardiac anomalies underwent coronary sinus cut-back technique with glutaraldehyde pericardial patch closure of ASD while re-routing pulmonary venous blood into left atrium except one who underwent Van Praagh technique. All patients were started on cardiac support infusions; Milrinone (5-10µg/kg/min) and Dobutamine (2-20µg/kg/min) intra-operatively and transferred to Cardiothoracic ICU for elective ventilation, cardiac support and monitoring.    Results  The study included a total of 65 patients whereby male accounted for 56.9% and female 43.1% (Table 1). Total anomalies were found in 39 patients accounting for 60% and 26 patients (40%) with partial anomalies (Tables 2 & 3). Mean age of presentation was 21.6 months (variance = 832.71, SD=28.86) for TAPVC and 135.2 months (variance = 27567.36, SD=166.03) for PAPVC (p = 0.0031). Among patients with partial anomalies 17 (65.4%) had involvement of one pulmonary vein (right superior pulmonary vein), 8 (30.8%) had involvement of two veins (right superior and right inferior pulmonary veins) and only 1 patient (3.8%) had involvement of three pulmonary veins (two from right side and one from left side draining into innominate vein ). Majority of partial anomalies were associated with sinus venosus ASD (84.6%) and only 7.7% were associated with posterior ASD and 7.7% ostium secundum ASD (Table 2).  Among all patients with TAPVC 17 (43.6%) had supra-cardiac type, 14 (35.9%) had intra-cardiac type, 2 (5.1%) had infra-cardiac type and 6 (15.4%) had a mixed type (Table 3). TAPVC was associated with ostium secundum ASD in 20 patients accounting for 51.3%. Other rare anomalies associated with TAPVC included LSVC, PDA and VSD (Table 4). Pulmonary hypertension was found in 29 out of 39 (74.4%) patients with TAPVC  compared to 3 out of 26 (11.5%) patients with PAPVC (X2 = 24.63, p=0.000001).There were 6 deaths from TAPVC group compared to none from PAPVC.   20  East and Central African Journal of Surgery Volume 12 Number 2      November /December 2007  All deaths were associated with severe pulmonary hypertension and 5 out of 6 were 2 months old. These deaths occurred within 48 hours of surgery except one that occurred on the seventh day and these were due to multiple organ failure due to low cardiac output syndrome and severe pulmonary hypertension. No patient reported to have post-operative pulmonary vein obstruction.   Table 1. Age / Sex Distribution.  Age group (months)  Male (%)     Female (%) Total (%) 0-12 19 11 30 (46.2) 13-60   6 13 19 (29.2) > 61 12   4 16 (24.6) Total 37 (56.9) 28 (43.1) 65 (100)   Table 2. Type of ASD associated with PAPVC  Type of ASD Number % Sinus venosus ASD 22 84.6 Posterior ASD 2 7.7 Ostium secundum ASD 2 7.7  Total 26 100  Table 3. Type of TAPVC  Type of TAPVC Number % Supracardiac 17 43.6 Intracardiac 14 35.9 Infracardiac 2 5.1 Mixed 6 15.4  Total 39 100  Table 4. Anomalies  associated  with TAPVC  Type of Anomaly  No.  (%)         ASD 20  (51.3) Left Superior Vena Cava (LSVC)   2   (5.2) Patent Dactus Arteriosus (PDA)   1   (2.6) PDA + ASD   1   (2.6) PDA + LSVC   1   (2.6) Ventricular Septal Defect (VSD)   1   (2.6) Total 26 (66.7) Thirteen patients (33.3%) had no associated anomalies.  Discussion   Anomalous Pulmonary Venous Connection present early in life. Lamb RK et al12) studied on APVC and found that 87.5% of all patients were less than 1 year old at time of operation. Our study revealed that 46.2% of all patients were 1-year old or less and TAPVC presenting early in life than PAPVC (mean age, 21.6 versus 135.2 months respectively, p = 0.0031). Male prepondence has been found in many studies1,7,12,13 as well as in our study.  TAPVC occurs most common than the PAPVC. Snellen HA et al (1) studied on the patterns of APVC and found that TAPVC was 10 times more common than PAPVC. PAPVC mainly involves right pulmonary vein(s) and commonly associated with sinus venosus ASD1,3. NM Ammash et al6 while studying on the diagnosis of the PAPVC found that 81.4 % of the patients involved right pulmonary veins, 16.3 % involved left side and 2.3% was bilateral. CHA Jost et al3 when evaluating a long term post-operative outcome for 115 patients with sinus venosus ASD found that 97% were associated with APVC. Our study revealed that TAPVC accounts for 60% of all patients compared to 40% for PAPVC and among all patients with PAPVC 96.2% involved the right pulmonary veins. Supra-cardiac and intra-cardiac types of TAPVC are the commonest both accounting for 69.5% to 89.7% 4,10,12,13 which is comparable with our findings of 79.5%. Infra-cardiac and mixed types accounts for 10.0% to 31.3% and 20.5% in our study.  Definitive diagnosis for APVC is mainly made by Trans-Thoracic Echocardiography (TTE) and Trans-Esophageal Echocardiography (TEE) is considered for sinus venosus ASD or unexplained dilated right side of the heart by TTE6,7,8.  PAPVC carries a low morbidity and mortality of less than 0.9% compared to TAPVC which carries a mortality of 7 to 31%4,5,10,11,12,13,14, 15. 21  East and Central African Journal of Surgery Volume 12 Number 2      November /December 2007  TAPVC commonly is associated with ASD13 and when associated with a complex intra-cardiac anomaly the mortality increases to 52%16.The commonest anomaly associated with TAPVC in our study is ASD accounting for 51.3%. Overall mortality rate from our study was 9.2%; all deaths from TAPVC group making a mortality of 15.4% for TAPVC group. Pulmonary hypertension occured significantly in patients with TAPVC compared to PAPVC (X2 = 24.63, p=0.000001) and subsequently prolonged duration of mechanical ventilation and ICU stay.   Mortality in our study was attributed to pulmonary hypertension and low cardiac output syndrome. Choudhary et al 4 found that the predictors for early death in TAPVC included young age at operation, need for emergency surgery, malnutrition, pulmonary vein obstruction, pulmonary hypertension and low cardiac output syndrome.   There was no patient who developed pulmonary vein obstruction or cardiac arrhythmias in our study. Prevalence of pulmonary vein obstruction has been reported in other studies ranging from 6% to 14% occurring between 2 to 12 weeks or longer following surgery for TAPVC12,13,14,15. Pulmonary vein obstruction carries a high morbidity and mortality of more than 27% for re-operation15. Cardiac arrhythmias occurs very late following surgery for TAPVC  and therefore a long term follow-up for these patients is recommended even when asymptomatic11,17.  Conclusion TAPVC commonly present early in life and is associated with ASD and pulmonary hypertension. It carries a high morbidity and mortality. Accurate diagnosis and early surgical correction improves the surgical outcome.   References  1. Snellen HA, van Ingen HC, Hoefsmit EC. Patterns of anomalous pulmonary venousdrainage. Circulation 1968; 38: 45 – 63 2. VA Ferrari, CH Scott, GA Holland, L Axel, MJ Sutton. Resonance angiography and imaging in the diagnosis of partial anomalous pulmonary venous drainage. J Am Coll Cardiol 2001; 37: 1120 – 1128 3. CHA Jost, HM Connolly, GK Danielson, KR Bailey, HV Schaff, WK Shen, CA Warnes, JB Seward, FJ Puga, AJ Tajik. Sinus Venosus Atrial Septal Defect: Long-Term Postoperative Outcome for 115 Patients. Circulation 2005; 112: 1953 – 1958 4. SK Choudhary, A Bhan, R Sharma, B Airan, V Devagourou, A Saxena, SS Kothari, P Venugopal. Total Anomalous Pulmonary Venous Connection: Surgical Experience in Indians. Indian Heart J 2001; 53:754 – 760  5. N Sinzobahamvya, C Arenz, AM Brecher, HC Blaschczok, AE Urban. Early and long-term results for correction of total anomalous pulmonary venous drainage in neonates and infants. Eur J Cardiothorac Surg 1996; 10: 433 - 438  6. NM Ammash, JB Seward, CA Warnes, HM Connolly, PW O’Leary, GK Danielson. Partial Anomalous Pulmonary Venous Connection: Diagnosis by Trans Esophageal Echocardiography. J Am Coll Cardiol 1997; 29: 1351 – 1358. 7. RD Pascoe, JK Oh, CA Warnes, GK Danielson, AJ Tajik, JB Seward. Diagnosis of Sinus Venosus Atrial Septal Defect with Trans-Esophageal Echocardiography. Circulation 1996; 94: 1049 – 1055 8. Huhra JC, Gutgessell HP, Nihill MR. Cross Sectional Echocardiographic diagnosis of TAPVC. Br Heart J 1985; 53: 525 – 534 9. LD Allan, GK Sharland. The echocardiographic diagnosis of totally anomalous pulmonary venous 22  East and Central African Journal of Surgery Volume 12 Number 2      November /December 2007  connection in the fetus. Heart 2001; 85: 433 -437.  10. SK Choudhary, A Bhan, R Sharma, A Mathur, B Airan, A Saxena, SS Kothari, R Juneja, P Venugopal. Repair of total anomalous pulmonary venous connection in infancy: experience from a developing country. Ann Thorac Surg 1999; 68: 155 –159  11. Total Anomalous Pulmonary Venous Connection: An Analysis of Current Management Strategies in a Single Institution. Ann Thorac Surg 2005; 79: 596 -606 12. RK Lamb, SA Queresh, JL Wilkinson, R Arnold, CR West, DI Hamilton. Total anomalous pulmonary venous drainage: Seventeen-year surgical experience. J Thorac & Cardiovasc Surg 1988; 96: 368 – 375 13. NM Noori, M Meraji, MHK Mohamed, H Mottaghi, SM Alizadeh, GM Sadeghi.Surgical outcome for correction of TAPVC in children. Iranian Heart J 2001; 2: 15 –18 14. JAJ Hyde, O Stumper, MJ Barth, JGC Wright, ED Silove, JV de Giovanni, WJ Brawn, B Sethia. Total anomalous                      pulmonary venous connection:outcome of  surgical correction and management of recurrent venous obstruction. Eur J Cardiothorac Surg 1999; 15: 735 - 741    15. F Lacour-Gayet, J Zoghbi, AE Serraf, E Belli, D Piot, C Rey, F Marcon, J Bruniax, C Planche. Surgical Management of Progressive Pulmonary Venous Obstruction after  Repair of Total Anomalous Pulmonary Venous Connection. J Thorac Cardiovasc Surg 1999; 117: 679 - 687   16. CA Caldarone, HK Najm, M Kadletz, JF Smallhorn, RM Freedom, WG Williams, JG Coles. Surgical management of total anomalous pulmonary venous drainage: impact of coexisting cardiac anomalies. Ann Thorac Surg 1998; 66: 1521- 1526  17. A Saxena, LV Fong, RK Lamb, JL Monro, DF Shore, BR Keeton. Cardiac arrhythmias after surgical correction of total anomalous pulmonary venous connection: Late follow-up. Pediatr Cardiol 1991; 12: 89 – 91                 

Patterns of anomalous pulmonary venous connection as seen at Care Hospital, Hyderabad - India

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Modified blalock-taussig shunt in palliative cardiac surgery

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