Blue babies: When to intervene
http://www.100md.com
《美国医学杂志》
Escorts Heart Institute & Research Centre, New Delhi, India
Abstract
Cyanotic congenital heart disease is an important cause of morbidity and mortality in neonates, infants and children. Presently facilities for treatment for most of these cases are available in many centres in India also. The most important factor is awareness, complete correct diagnosis and timely intervention either palliative or corrective. In admixture lesions very early intervention is needed so as to avoid development of pulmonary vascular obstructive disease which develops early in these cases. Some of the patients of reduced pulmonary blood flow may need aggressive intervention in neonatal period. The patient of tetralogy of Fallot should have surgical correction before their first birthday. Due to great variation in the anatomical substrate and natural history each case needs complete meticulous evaluation. Later a plan of management keeping in view the natural history of the uncorrected lesion should be made.
Keywords: Cyanotic congenital heart disease; Morbidity; Mortality; Intervention
Abbreviations
PDA Patient ductus arteriosus
PGE1 Prosta glandin E1
PFO Patent Foramen Ovale
BT Blalock Taussig
SVC Superior vena cava
PA Pulmonary artery
TOF Tetralogy of Fallot
CHF Congestive heart failure
dTGA dextro transposition of great arteries
L levo
VSD Ventricular Septal defect
BAS Balloon Atrial Septostomy
PAH Pulmonary arterial Hypertension
Blue babies are doomed babies is a myth. With the advancing technologies and expertise in the field of pediatric cardiac sciences, a large percentage of cyanotic heart defects in childhood can either be corrected completely or adequately palliated. This requires teamwork of obstetrician, neonatologist, pediatrician, pediatric cardiologist, pediatric cardiac surgeon and his team. With the availability of high-resolution echocardiographic systems, the diagnostic work up may start in fetal life in some cases. In the present era most of the diagnostic work up is done by echocardiographic evaluation supplemented by spiral CT/ MRI in some cases. Cardiac catheterisation and angiocardiography are mostly needed in some selected cases and those cases who need interventional procedure as a preparation to surgery, palliative or curative.
The cynotic heart diseases can be discussed under following groups.
(1) Cyanosis is dependant on right to left shunt due to pulmonary stenosis or atresia.
(2) Cyanosis due to abnormal connections of great vessels, pulmonary veins or absence of cardiac septa - admixture lesions.
(3) Cyanosis is due to pulmonary arterial hypertension causing right to left shunt associated with pulmonary venous hypertension.
(4) Cyanosis is due to right to left shunt due to elevated fixed pulmonary vascular resistance (Eisenmenger syndrome).
(5) Miscellaneous
Group I
1. Pulmonary atresia with intact septum
They are very sick patients and present in neonatal life with severe cyanosis and congestive heart failure. Their pulmonary blood and survival is dependant of the patency of the PDA. As such as soon as they are detected they should be started on PGE1 infusion to keep the ductus arteriosus patent. Subsequently a detailed echocardiographic evaluation to assess the atrial communication, right ventricular size, inflow, cavity, outflow, type of atresia - membranous/muscular, dependency of coronary circulation should be done. Patients in whom the right ventricle is hypoplastic and coronary circulation is dependant on right ventricle will be candidates for early Blalock Taussig shunt. If they are associated with restrictive PFO this needs to be enlarged also.
After shunt these patients will follow the pathway of univentricular correction i.e. Glenn's shunt (SVC-PA) and take down of BT shunt by one year of age and total cavo pulmonary connection by 3-4 years of age.[1],[2]
Those who do not have RV dependant coronary circulation usually have better right ventricular cavity. If they have membranous pulmonary atresia, they will be benefited by performing radio frequency guided pulmonary valvar perforation and balloon dilation of the pulmonary valve.[3],[4] Patients with non-dependant coronary circulation and muscular pulmonary atresia, if have associated mild right ventricular hypoplasia, are taken up for surgical right ventriculotomy. They may or may not need additional BTshunt. Later depending on the saturation and right ventricular size further surgery is planned. If they have small right ventricle one and a half-ventricular repair is planned.
2. Tetralogy of Fallot physiology (TOF)
Patients with TOF can rarely present in neonatal period if the right ventricular out-flow obstruction is severe and there is no PDA. Usually they present after neonatal period generally in early infancy.
In patients with TOF, if the anatomy is good i.e. adequate sized pulmonary annulus and pulmonary arteries, single VSD, normal coronary anatomy, the ideal treatment is total correction at the age of 6 months if patient is stable.[5],[6] If the patient is below 6 months and has repeated spells, not controlled with the treatment of precipitating factors and beta-blockers, or the saturation is below 70%, Hb above 18 gm%, PCV above 60 or if the pulmonary arteries are small, the child needs palliation with BT shunt or percutaneous balloon dilation of the right ventricular outflow at presentation. In stable patients, if the pulmonary annulus is small but the other anatomy is adequate the elective total correction could be delayed to 9-12 months[7]. Patients who have undergone palliation in infancy should be evaluated one year later electively for total correction.
In patients with TOF physiology, with pulmonary arteries posterior to aorta and adequate anatomy to give biventricular correction e.g. double outlet right ventricle (DORV) with malposed great vessels or transposition (D or L) with ventricular septal defect with PS, initial palliation may be needed as discussed under TOF. Subsequently, total correction with conduit between right ventricle and pulmonary artery and VSD closure is done around the age of 4 years.[8]
In patients with tricuspid atresia or univentricular physiology with pulmonary stenosis, biventricular correction is not possible. These patients will be candidates for univentricular repair around 3-4 years of age wherein the systemic venous blood is directly routed to pulmonary arteries by Fontan's operation or total caval pulmonary connection. [9],[10],[11],[12] In case they need palliation earlier than this, it can be done by doing B.T. shunt (before 5-6 months) or Glenn's shunt after 5-6 months. The indications for palliation are same as discussed under TOF.
Critical PS with TR
Some patients of isolated critical valvular PS present with cyanosis due to right to left atrial shunt generally in newborn or infancy but occasionally later also. They are usually associated with tricuspid regurgitation and CHF. These patients need emergency balloon dilation of the pulmonary valve with very good results.[13] In some cases the dilation is done in staged manner and some of them may need repeat balloon dilation.
Group II
Cyanosis with Increased Pulmonary Blood Flow Admixture Lesions
I. D transposition of great vessels (dTGA) : dTGA is the commonest lesion is this group. Babies with dTGA and intact ventricular septum present with cyanosis, CHF and hypoxia in the newborn period. These babies are very sick and should be started on PGE1 infusion with an infusion pump in the dose of 0.01 ugm/kg/min.[14] During the infusion careful watch on respiration should be kept. One should be equipped to handle apnoea. The dose of PGE1 is to be adjusted according to the response. Other supportive measures should be started and the baby should be transported to a centre where balloon atrial septostomy (BAS) can be done to increase mixing.[15],[16] BAS can even be done under echocardiographic guidance also. After adequate septostomy prostaglandin infusion can be stopped. Babies with dTGA with intact septum should undergo arterial switch operation before 4 weeks of life.[17],[18],[19] If the babies present later, they should have Senning's operation (rerouting at atrial level) at 3 months of age.[20],[21] Babies with dTGA and VSD need aggressive decongestive therapy and balloon atrial septostomy to decompress the left atrium and improve CHF. The arterial switch operation and VSD closure are done between 4-8 weeks of life.[17]
Patients with increased pulmonary blood flow and univentricular physiology, where single ventricle repair is the only option, should have pulmonary artery banding at 4-8 weeks of life, the time when CHF starts appearing to prevent the effects of excessive pulmonary blood flow i.e. pulmonary hypertension, ventricular dilation or ventricular outlet obstruction.[22],[23] Later systemic venous rerouting operation after 2 years of age is done.[24] Some of them may need Glenn's shunt in the interim period.
2. Total anomalous pulmonary venous connection : Patients with this anomaly need surgical correction at presentation.[25]
3. Persistent truncus arteriosus: Patients should have surgical correction i.e. closure of the VSD and right ventricle to pulmonary artery conduit at 4-6 weeks of life.[26] The first appearance of CHF in the form of tachypnoea can be taken as an indication of fall in pulmonary vascular resistance and optimal time for corrective surgery.
In case corrective surgery can not be planned due to logistic reasons they should undergo pulmonary arterial banding at 4-6 weeks and later corrective surgery.[27] In late presenters very careful assessment of operability is needed before planning surgery.
Group III
Cyanosis with pulmonary venous hypertension
(i) TAPVC with obstruction is the commonest lesion in this group. The presentation is uusually in neonatal period with severe PAH, cyanosis and respiratory distress. They need urgent supportive measures with ventilatory support. Careful assessment should be done for the site of obstruction. If the obstruction is at the level of PFO, these patients can be stabilised by doing balloon atrial septostomy and later corrective surgery can be electively done. If the obstruction is at the site of drainage or in the channel of drainage, emergency surgery is needed.[25]
(ii) Hypoplastic left heart syndrome. In cases of classic hypoplastic left heart with mitral, aortic atresia / hypoplasia we discuss with the family the options of repeated surgeries with not such good outcome and heart transplant[28],[29]. As these are not easily feasible at present in our country, we do not encourage them to take a surgical option.
Group IV
Elevated Fixed Pulmonary vascular resistance and R-L shunt (Eisenmenger Syndrome)
The best treatment for this group is prevention. If shunt lesion are timely managed one should not see these cases. Unfortunately one still sees these cases and can not offer any definitive therapy.[30],[31]
Group V
Miscellaneous
(a) Ebsteins anomaly of the tricuspid valve
Newborn with severe type of Ebstein's anomaly of the tricuspid valve may present with CHF, tricuspid regurgitation and right to left atrial shunt. These patients can usually be stabilised with medical treatment, as they tend to improve with fall of pulmonary vascular resistance. Corrective or palliative surgery is planned according to the degree of tricuspid valve displacement electively. In some cases the tricuspid valve can be repaired.[32] If the tricuspid valve can not be repaired they follow the pathway of univentricular repair i.e. Glenn's shunt and if needed followed by completion of Fontan.
The timing is dependent of the clinical status of the patient. If there is persistant cyanosis, significant cardiomegally and CHF early surgical intervention is needed.
b. Systemic venous connection to left atrium
Left SVC to left artium is a rare anomaly in isolation.[33] These patients need elective surgery by 3-4 years of age. Total anomolous systemic venous connection is an extremely rare anomoly when all the systemic veins drain to the left atrium, these patients need surgical correction at presentation.[34]
c. Mimickers of cyanotic CHD in the Newborn
(i) Some infants present with cyanosis due to right to left shunt at atrial level with persistent pulmonary hypertension. The most important role of the pediatric cardiologist is to rule out any treatable cause for PAH. They are best managed by neonatologist with ventilatory and other supportive measures.
(ii) Severe chest infection may result in cyanosis in newborn and infants. Proper evaluation and hyperoxic test easily rule out CHD being the cause of cyanosis.
Thus it is obvious that most of the congenital cyanotic cardiac lesions can be offered catheter based or surgical correction/palliation. The most crucial step is to arrive at a complete correct diagnosis as soon as possible and offer the best mode of therapy timely before severe malnutrition, ventricular dysfunction, pulmonary vascular changes, hypoxia and polycythemia related problems set in. Timely correction results in lesser post operative mortality, morbidity and good long term results. High index of suspicion and aggressive approach is necessary for fruitful outcome.
References
1. Rodhard S, Wagner D. Bypassing the right ventricle. Proc Soc Exp Bio Med 1949; 71: 69.
2. Glenn WWL, Ordway NK, Talner NS et al. Circulatory bypass of the right side of heart VI; shunt between superior vena cava and distal right pulmonary artery; report of clinical application in 38 cases. Circulation 1965; 31: 172-189.
3. Gibbs JL, Black Bwon ME, Ozum O et al. Laser valvotomy and balloon valvuloplasty for pulmonary atresia and intact ventricular septum; Five year follow up. Heart 1997; 77(2)225-224.
4. Qureshi SA, Rosenthal E, Tynan M et al. Transcatheter laser assisted balloon pulmonary valve dilation in pulmonary valve atresia. Am J Cardiol 1991; 67: 428-443.
5. Castaneda AR, Freed MO, Williams RG et al. Repair of tetralogy of Fallot in infancy, early and late results. J Thorac Cardiovasc Surg 1977; 74: 372-381.
6. Kirklin JW, Black Stone EH, Kirklin JK et al. Surgical results and protocols in specture of tetralogy of Fallot. Ann Sur 1982; 198: 251-265.
7. Kirklin JK, Kirklin JW, Pacifico AO. Transannular outflow patching for tetralogy, indications and results. Thorac Cardiovasc Surg 1990; 2: 61-69.
8. Pacifico A, Kerklin JW et al. Complex Congenital Malformations Surgical Treatment of Double Outlet. Right Ventricle. In: Kerklin JW (ed). Advanced Cardiovascular Surgery. New York; Crane and Stratlon 1973:57.
9. Choussat A, Fontan F, Besse P et al. Selection criteria for Fontan Procedure. In Anderson FH, Shinebourne EA, eds. Pediatric Cardiology . White Plains, New York; Churchill Living Stone, 1978; 559-566.
10. Gale AW, Danielson GK, Mc Goon DC, Mair DD. Modified Fontan operation for univentricular heart and complicated congenital lesions. J Thorac Cardiovasc Surg 1979; 78: 831-838.
11. Kawashima Y, Kitamure S, Satsude H et al. Total cavopulmonary shunt operation in complex cardiac anomalies. A new operation. J Thorac Cardiovasc Surg 1984; 87: 74-81.
12. Pearl JM, Laks H, Stein DG et al. Total Cavopulmonary anastomosis versus conventional modified Fontan Procedure. Ann Thorac Surg 1991; 52: 189.
13. Colt AM, Perry SR, Lock JE et al. Balloon dilation of critical valvar pulmonary stenosis in the first month of life. Cath Cardiovas. Diagnosis 1995; 34: 23-28.
14. Eitzke A Suppanch. Use of prostaglandin E in management of transposition of great arteries before balloon atrial septostomy. Br Heart J 1983; 49: 341-344.
15. Rashkind WJ, Miller WW. Creation of an atrial septal defect without thoractomy. A palliative approach to complete transposition of great arteries. JAMA 1966; 96: 113-114.
16. Baker e.j. Allan LA, Tynan MJ. Balloon atrial septostomy in the neonatal intensive care unit. Br Heart J 1984; 51: 337-338.
17. Kaya MP. Anatomic correction of transposition of great arteries. Mayo Clin Proc 1975; 50: 638-640.
18. Jonas RA, Gigtia TM, Sanders PS et al. Rapid two stage arterial switch for transposition of great arteries and intact interventricular septum beyond neonatal period. Circulation 1989; 80(suppl. 1): 203-208.
19. Castaneda AR, Norwood WI, Jonas RA, Colan SD et al. Transposition of great arteries and intact ventricular septum, anatomical repair in the neonate. Ann Thorac Surg 1984; 38: 438-443.
20. Mahoney L Turleyk, Ebert PA, Heymann MA. Long term results after atrial repair of transposition of great arteries in early infancy. Circulation 1982; 66: 253-288.
21. Senning A. Surgical correction of transposition of great vessels. Surgery 1959; 46: 966-980.
22. Malac I, Saver U, Sterm H et al. The influence of pulmonary artery banding on outcome after Fontan operation. J Thorac Cardiovasc Surg 1992; 104(3): 743-747.
23. Oldham NH (Hr.), Kakej GS, Jarmakani MA et al. Pulmonary artery banding in infants with complex congenital heart defects. Ann Thorac Surg 1972; 13: 342.
24. Stein DG, Laks H, Drinkwater DC et al. Results of total cavo pulmonary connection in the treatment of patients with a functional single ventricle. J Thorac Cardiovasc Surg 1991; 102(2): 280-286.
25. Raisher B, Grant JW, Martin TL et al. Complete repair of total anomalous pulmonary venous connection in infancy. J Thorac Cardiovasc Surg 1992; 104: 443.
26. Hariley F, Heinemann MK, Jonas RA et al. Repair of truncus arteriosus in the neonate. J Thorac Cardiovasc Surg 1993; 105: 1047-1056.
27. Pearl JM, Laks H, Drinkwater DC (Jr.) et al. Repair of truncus arteriosus in infancy. Ann Thorac Surg 1991; 52(4): 780-786.
28. Bu' Lock FA, Stumper O, Jagtap R, Silove ED, De Giovanni JV, Wright JG et al. Surgery for infants with a hypoplastic systemic ventricle and severe outflow obstruction: early results with a modified norwood procedure. Br Heart J 1995; 73: 456
29. Backer CL, Idriss FS, Zales VR, Mavroudis C, Cardiac transplantation hy hypoplastic left heart syndrome: a modified technique. Ann Thorac Surg 1990; 50: 894.
30. Brammell HL. The Eisenmenger Syndrome. Aclinical and physiologic reappraisal. Am J Cardiol 1971; 28: 679-692.
31. Vongpatanasin W, Brickner E, Hills D. Eisenmenger Syndrome in adults. Ann of Int Med 1998; 128: 745-755.
32. Theodoro DA, Danielson GK, Kiziltan HT, Dziscoll DJ, Mair DD, Warnes CA et al. Surgical management of Ebsteins anomaly - a 25 year experience. Circulation 1997: 96-507.
33. Davis WH, Jordoan FR, Swyman HW. persistant left superior vena cava draining into the left atrium as inisolated anomaly. Am Heart J 1959; 57: 616-622.
34. Pearl WR, Spicer MJ. Total anomalous systemic venous return. South Med J 1980; 73: 259-261.(Shrivastava Savitri)
Abstract
Cyanotic congenital heart disease is an important cause of morbidity and mortality in neonates, infants and children. Presently facilities for treatment for most of these cases are available in many centres in India also. The most important factor is awareness, complete correct diagnosis and timely intervention either palliative or corrective. In admixture lesions very early intervention is needed so as to avoid development of pulmonary vascular obstructive disease which develops early in these cases. Some of the patients of reduced pulmonary blood flow may need aggressive intervention in neonatal period. The patient of tetralogy of Fallot should have surgical correction before their first birthday. Due to great variation in the anatomical substrate and natural history each case needs complete meticulous evaluation. Later a plan of management keeping in view the natural history of the uncorrected lesion should be made.
Keywords: Cyanotic congenital heart disease; Morbidity; Mortality; Intervention
Abbreviations
PDA Patient ductus arteriosus
PGE1 Prosta glandin E1
PFO Patent Foramen Ovale
BT Blalock Taussig
SVC Superior vena cava
PA Pulmonary artery
TOF Tetralogy of Fallot
CHF Congestive heart failure
dTGA dextro transposition of great arteries
L levo
VSD Ventricular Septal defect
BAS Balloon Atrial Septostomy
PAH Pulmonary arterial Hypertension
Blue babies are doomed babies is a myth. With the advancing technologies and expertise in the field of pediatric cardiac sciences, a large percentage of cyanotic heart defects in childhood can either be corrected completely or adequately palliated. This requires teamwork of obstetrician, neonatologist, pediatrician, pediatric cardiologist, pediatric cardiac surgeon and his team. With the availability of high-resolution echocardiographic systems, the diagnostic work up may start in fetal life in some cases. In the present era most of the diagnostic work up is done by echocardiographic evaluation supplemented by spiral CT/ MRI in some cases. Cardiac catheterisation and angiocardiography are mostly needed in some selected cases and those cases who need interventional procedure as a preparation to surgery, palliative or curative.
The cynotic heart diseases can be discussed under following groups.
(1) Cyanosis is dependant on right to left shunt due to pulmonary stenosis or atresia.
(2) Cyanosis due to abnormal connections of great vessels, pulmonary veins or absence of cardiac septa - admixture lesions.
(3) Cyanosis is due to pulmonary arterial hypertension causing right to left shunt associated with pulmonary venous hypertension.
(4) Cyanosis is due to right to left shunt due to elevated fixed pulmonary vascular resistance (Eisenmenger syndrome).
(5) Miscellaneous
Group I
1. Pulmonary atresia with intact septum
They are very sick patients and present in neonatal life with severe cyanosis and congestive heart failure. Their pulmonary blood and survival is dependant of the patency of the PDA. As such as soon as they are detected they should be started on PGE1 infusion to keep the ductus arteriosus patent. Subsequently a detailed echocardiographic evaluation to assess the atrial communication, right ventricular size, inflow, cavity, outflow, type of atresia - membranous/muscular, dependency of coronary circulation should be done. Patients in whom the right ventricle is hypoplastic and coronary circulation is dependant on right ventricle will be candidates for early Blalock Taussig shunt. If they are associated with restrictive PFO this needs to be enlarged also.
After shunt these patients will follow the pathway of univentricular correction i.e. Glenn's shunt (SVC-PA) and take down of BT shunt by one year of age and total cavo pulmonary connection by 3-4 years of age.[1],[2]
Those who do not have RV dependant coronary circulation usually have better right ventricular cavity. If they have membranous pulmonary atresia, they will be benefited by performing radio frequency guided pulmonary valvar perforation and balloon dilation of the pulmonary valve.[3],[4] Patients with non-dependant coronary circulation and muscular pulmonary atresia, if have associated mild right ventricular hypoplasia, are taken up for surgical right ventriculotomy. They may or may not need additional BTshunt. Later depending on the saturation and right ventricular size further surgery is planned. If they have small right ventricle one and a half-ventricular repair is planned.
2. Tetralogy of Fallot physiology (TOF)
Patients with TOF can rarely present in neonatal period if the right ventricular out-flow obstruction is severe and there is no PDA. Usually they present after neonatal period generally in early infancy.
In patients with TOF, if the anatomy is good i.e. adequate sized pulmonary annulus and pulmonary arteries, single VSD, normal coronary anatomy, the ideal treatment is total correction at the age of 6 months if patient is stable.[5],[6] If the patient is below 6 months and has repeated spells, not controlled with the treatment of precipitating factors and beta-blockers, or the saturation is below 70%, Hb above 18 gm%, PCV above 60 or if the pulmonary arteries are small, the child needs palliation with BT shunt or percutaneous balloon dilation of the right ventricular outflow at presentation. In stable patients, if the pulmonary annulus is small but the other anatomy is adequate the elective total correction could be delayed to 9-12 months[7]. Patients who have undergone palliation in infancy should be evaluated one year later electively for total correction.
In patients with TOF physiology, with pulmonary arteries posterior to aorta and adequate anatomy to give biventricular correction e.g. double outlet right ventricle (DORV) with malposed great vessels or transposition (D or L) with ventricular septal defect with PS, initial palliation may be needed as discussed under TOF. Subsequently, total correction with conduit between right ventricle and pulmonary artery and VSD closure is done around the age of 4 years.[8]
In patients with tricuspid atresia or univentricular physiology with pulmonary stenosis, biventricular correction is not possible. These patients will be candidates for univentricular repair around 3-4 years of age wherein the systemic venous blood is directly routed to pulmonary arteries by Fontan's operation or total caval pulmonary connection. [9],[10],[11],[12] In case they need palliation earlier than this, it can be done by doing B.T. shunt (before 5-6 months) or Glenn's shunt after 5-6 months. The indications for palliation are same as discussed under TOF.
Critical PS with TR
Some patients of isolated critical valvular PS present with cyanosis due to right to left atrial shunt generally in newborn or infancy but occasionally later also. They are usually associated with tricuspid regurgitation and CHF. These patients need emergency balloon dilation of the pulmonary valve with very good results.[13] In some cases the dilation is done in staged manner and some of them may need repeat balloon dilation.
Group II
Cyanosis with Increased Pulmonary Blood Flow Admixture Lesions
I. D transposition of great vessels (dTGA) : dTGA is the commonest lesion is this group. Babies with dTGA and intact ventricular septum present with cyanosis, CHF and hypoxia in the newborn period. These babies are very sick and should be started on PGE1 infusion with an infusion pump in the dose of 0.01 ugm/kg/min.[14] During the infusion careful watch on respiration should be kept. One should be equipped to handle apnoea. The dose of PGE1 is to be adjusted according to the response. Other supportive measures should be started and the baby should be transported to a centre where balloon atrial septostomy (BAS) can be done to increase mixing.[15],[16] BAS can even be done under echocardiographic guidance also. After adequate septostomy prostaglandin infusion can be stopped. Babies with dTGA with intact septum should undergo arterial switch operation before 4 weeks of life.[17],[18],[19] If the babies present later, they should have Senning's operation (rerouting at atrial level) at 3 months of age.[20],[21] Babies with dTGA and VSD need aggressive decongestive therapy and balloon atrial septostomy to decompress the left atrium and improve CHF. The arterial switch operation and VSD closure are done between 4-8 weeks of life.[17]
Patients with increased pulmonary blood flow and univentricular physiology, where single ventricle repair is the only option, should have pulmonary artery banding at 4-8 weeks of life, the time when CHF starts appearing to prevent the effects of excessive pulmonary blood flow i.e. pulmonary hypertension, ventricular dilation or ventricular outlet obstruction.[22],[23] Later systemic venous rerouting operation after 2 years of age is done.[24] Some of them may need Glenn's shunt in the interim period.
2. Total anomalous pulmonary venous connection : Patients with this anomaly need surgical correction at presentation.[25]
3. Persistent truncus arteriosus: Patients should have surgical correction i.e. closure of the VSD and right ventricle to pulmonary artery conduit at 4-6 weeks of life.[26] The first appearance of CHF in the form of tachypnoea can be taken as an indication of fall in pulmonary vascular resistance and optimal time for corrective surgery.
In case corrective surgery can not be planned due to logistic reasons they should undergo pulmonary arterial banding at 4-6 weeks and later corrective surgery.[27] In late presenters very careful assessment of operability is needed before planning surgery.
Group III
Cyanosis with pulmonary venous hypertension
(i) TAPVC with obstruction is the commonest lesion in this group. The presentation is uusually in neonatal period with severe PAH, cyanosis and respiratory distress. They need urgent supportive measures with ventilatory support. Careful assessment should be done for the site of obstruction. If the obstruction is at the level of PFO, these patients can be stabilised by doing balloon atrial septostomy and later corrective surgery can be electively done. If the obstruction is at the site of drainage or in the channel of drainage, emergency surgery is needed.[25]
(ii) Hypoplastic left heart syndrome. In cases of classic hypoplastic left heart with mitral, aortic atresia / hypoplasia we discuss with the family the options of repeated surgeries with not such good outcome and heart transplant[28],[29]. As these are not easily feasible at present in our country, we do not encourage them to take a surgical option.
Group IV
Elevated Fixed Pulmonary vascular resistance and R-L shunt (Eisenmenger Syndrome)
The best treatment for this group is prevention. If shunt lesion are timely managed one should not see these cases. Unfortunately one still sees these cases and can not offer any definitive therapy.[30],[31]
Group V
Miscellaneous
(a) Ebsteins anomaly of the tricuspid valve
Newborn with severe type of Ebstein's anomaly of the tricuspid valve may present with CHF, tricuspid regurgitation and right to left atrial shunt. These patients can usually be stabilised with medical treatment, as they tend to improve with fall of pulmonary vascular resistance. Corrective or palliative surgery is planned according to the degree of tricuspid valve displacement electively. In some cases the tricuspid valve can be repaired.[32] If the tricuspid valve can not be repaired they follow the pathway of univentricular repair i.e. Glenn's shunt and if needed followed by completion of Fontan.
The timing is dependent of the clinical status of the patient. If there is persistant cyanosis, significant cardiomegally and CHF early surgical intervention is needed.
b. Systemic venous connection to left atrium
Left SVC to left artium is a rare anomaly in isolation.[33] These patients need elective surgery by 3-4 years of age. Total anomolous systemic venous connection is an extremely rare anomoly when all the systemic veins drain to the left atrium, these patients need surgical correction at presentation.[34]
c. Mimickers of cyanotic CHD in the Newborn
(i) Some infants present with cyanosis due to right to left shunt at atrial level with persistent pulmonary hypertension. The most important role of the pediatric cardiologist is to rule out any treatable cause for PAH. They are best managed by neonatologist with ventilatory and other supportive measures.
(ii) Severe chest infection may result in cyanosis in newborn and infants. Proper evaluation and hyperoxic test easily rule out CHD being the cause of cyanosis.
Thus it is obvious that most of the congenital cyanotic cardiac lesions can be offered catheter based or surgical correction/palliation. The most crucial step is to arrive at a complete correct diagnosis as soon as possible and offer the best mode of therapy timely before severe malnutrition, ventricular dysfunction, pulmonary vascular changes, hypoxia and polycythemia related problems set in. Timely correction results in lesser post operative mortality, morbidity and good long term results. High index of suspicion and aggressive approach is necessary for fruitful outcome.
References
1. Rodhard S, Wagner D. Bypassing the right ventricle. Proc Soc Exp Bio Med 1949; 71: 69.
2. Glenn WWL, Ordway NK, Talner NS et al. Circulatory bypass of the right side of heart VI; shunt between superior vena cava and distal right pulmonary artery; report of clinical application in 38 cases. Circulation 1965; 31: 172-189.
3. Gibbs JL, Black Bwon ME, Ozum O et al. Laser valvotomy and balloon valvuloplasty for pulmonary atresia and intact ventricular septum; Five year follow up. Heart 1997; 77(2)225-224.
4. Qureshi SA, Rosenthal E, Tynan M et al. Transcatheter laser assisted balloon pulmonary valve dilation in pulmonary valve atresia. Am J Cardiol 1991; 67: 428-443.
5. Castaneda AR, Freed MO, Williams RG et al. Repair of tetralogy of Fallot in infancy, early and late results. J Thorac Cardiovasc Surg 1977; 74: 372-381.
6. Kirklin JW, Black Stone EH, Kirklin JK et al. Surgical results and protocols in specture of tetralogy of Fallot. Ann Sur 1982; 198: 251-265.
7. Kirklin JK, Kirklin JW, Pacifico AO. Transannular outflow patching for tetralogy, indications and results. Thorac Cardiovasc Surg 1990; 2: 61-69.
8. Pacifico A, Kerklin JW et al. Complex Congenital Malformations Surgical Treatment of Double Outlet. Right Ventricle. In: Kerklin JW (ed). Advanced Cardiovascular Surgery. New York; Crane and Stratlon 1973:57.
9. Choussat A, Fontan F, Besse P et al. Selection criteria for Fontan Procedure. In Anderson FH, Shinebourne EA, eds. Pediatric Cardiology . White Plains, New York; Churchill Living Stone, 1978; 559-566.
10. Gale AW, Danielson GK, Mc Goon DC, Mair DD. Modified Fontan operation for univentricular heart and complicated congenital lesions. J Thorac Cardiovasc Surg 1979; 78: 831-838.
11. Kawashima Y, Kitamure S, Satsude H et al. Total cavopulmonary shunt operation in complex cardiac anomalies. A new operation. J Thorac Cardiovasc Surg 1984; 87: 74-81.
12. Pearl JM, Laks H, Stein DG et al. Total Cavopulmonary anastomosis versus conventional modified Fontan Procedure. Ann Thorac Surg 1991; 52: 189.
13. Colt AM, Perry SR, Lock JE et al. Balloon dilation of critical valvar pulmonary stenosis in the first month of life. Cath Cardiovas. Diagnosis 1995; 34: 23-28.
14. Eitzke A Suppanch. Use of prostaglandin E in management of transposition of great arteries before balloon atrial septostomy. Br Heart J 1983; 49: 341-344.
15. Rashkind WJ, Miller WW. Creation of an atrial septal defect without thoractomy. A palliative approach to complete transposition of great arteries. JAMA 1966; 96: 113-114.
16. Baker e.j. Allan LA, Tynan MJ. Balloon atrial septostomy in the neonatal intensive care unit. Br Heart J 1984; 51: 337-338.
17. Kaya MP. Anatomic correction of transposition of great arteries. Mayo Clin Proc 1975; 50: 638-640.
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