Palliative surgery of a patient with hypoplastic left heart syndrome and low body weight.
Abstract
Aim: to demonstrate a clinical observation of the use of hemodynamic treatment in a newborn with hypoplasia syndromeof the left heart.Methods. A newborn with a congenital heart disease that affects normal blood flow through the heart and is characterizedby critical underdevelopment of the left heart (left heart hyperplasia syndrome) underwent the Norwood procedure. Italso provides observation during the inter-stage period and information on the procedure of bidirectional cavopulmonaryanastomosis.Results. Hypoplasia syndrome of the left heart is 1.4-3.8% among all congenital heart defects and 16% among criticalcongenital heart defects. Mortality in large cardiac surgery centers currently does not exceed 15%. However, mortality amongpatients with low body weight is up to 51% after the first stage of palliative treatment. In our clinical case, we describe a childwith hypoplasia of the left heart and low body weight, as well as hemodynamic treatment (Norwood procedure, inter-stageperiod and bidirectional cavopulmonary anastomosis procedure).Conclusion. Palliative hemodynamic correction in patients with hypoplasia syndrome of the left heart and low bodyweight can be performed with good early and long-term results.
References
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3. Bove E.L., Lloyd T.R. Staged reconstruction for hypoplastic left heart syndrome. Contemporary results. // Ann Surg. 1996. Vol. 224. P. 387-394.
4. Curzon C.L., Milford-Beland S., Li J.S., et al. Cardiac surgery in infants with low birth weight is associated with increased mortality: analysis of the Society of Thoracic Surgeons Congenital Heart Database. // J Thorac Cardiovasc Surg 2008. Vol. 135. P. 546-551.
5. Forbess J.M., Cook N., Roth S.J., Serraf A., Mayer J.E. Jr, Jonas R.A. Ten-year institutional experience with palliative surgery for hypoplastic left heart syndrome. Risk factors related to stage I mortality. // Circulation 1995. Vol. 92 (9 Suppl). P. II262-266.
6. Gelehrter S., Fifer C.G., Armstrong A., Hirsch J., Gajarski R. Outcomes of hypoplastic left heart syndrome in low-birthweight patients. // Pediatr Cardiol 2011. Vol. 32. P. 1175-1181.
7. Hirsch J.C., Gurney J.G., Donohue J.E., Gebremariam A., Bove E.L., Ohye R.G. Hospital mortality for Norwood and arterial switch operations as a function of institutional volume. // Pediatr Cardiol. 2008. Vol. 29. P. 713-717.
8. Kalfa D., Krishnamurthy G., Duchon J., et al. Outcomes of cardiac surgery in patients weighing <2.5 kg: affect of patient-dependent and -independent variables. // J Thorac Cardiovasc Surg 2014. Vol. 148. P. 2499-2506.e1.
9. Kalfa D., Krishnamurthy G., Levasseur S., Najjar M., Chai P., Chen J., Quaegebeur J., Bacha E. Norwood Stage I Palliation in Patients Less Than or Equal to 2.5 kg: Outcomes and Risk Analysis. // Ann Thorac Surg. 2015. Vol. 100 (1). P. 167-173.
10. Ohye R.G., Sleeper L.A., Mahony L., et al. Pediatric Heart Network Investigators. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. // N. Engl. J. Med. 2010. Vol. 362. P. 1980-1992.
11. Pizarro C., Davis D.A., Galantowicz M.E., Munro H., Gidding S.S., Norwood W.I. Stage I palliation for hypoplastic left heart syndrome in low birth weight neonates: can we justify it? // Eur J Cardiothorac Surg. 2002. Vol. 21. P. 716-720.
12. Tweddell J.S., Sleeper L.A., Ohye R.G., et al. Pediatric Heart Network Investigators. Intermediate-term mortality and cardiac transplantation in infants with single-ventricle lesions: risk factors and their interaction with shunt type. J Thorac Cardiovasc Surg 2012;144:152–9. (https://www.jtcvs.org/article/S0022-5223(12)00018-9/abstract)
13. Ugonabo N, Hirsch-Romano J.C., Uzark K. The role of home monitoring in interstage management of infants following the Norwood procedure. // World J Pediatr Congenit Heart Surg. 2015. Vol. 6(2). P. 266-273.
14. Weinstein S., Gaynor J.W., Bridges N.D., et al. Early survival of infants weighing 2.5 kilograms or less undergoing first-stage reconstruction for hypoplastic left heart syndrome. // Circulation. 1999. Vol. 100 (19 Suppl). P. II167–70.
REFERENCES
1. Voitov A.V., Omelchenko A.Yu., Soinov I.A., Nichay N.R., Gorbatykh A.V., Kulyabin Yu.Yu., Arkhipov A.N., Bogachev-Prokofiev A.V. ., Karaskov A.M. The result of using a venous valve-containing allograft in the rehabilitation of pulmonary arteries in a newborn with atresia of the pulmonary artery and a defect in the interventricular septum: a clinical case. // Patologiya krovoobrashcheniya i kardiokhirurgiya. 2018.Vol. 22. No. 2. P. 68-72. DOI: 10.21688/1681-3472-2018-2-68-72 (in Russian)
2. Matyushov VN, Sinelnikov Yu.S., Soinov I.A., Kornilov I.A., Prokhorov S.N., Chashchin O.V., Ivantsov S.M. Analysis of the experience of surgical treatment of the common arterial trunk. // Byulleten' Sibirskogo otdeleniya Rossiyskoy akademii meditsinskikh nauk. 2014. Vol. 34. No. 1. P. 60-66. (in Russian)
3. Bove E.L., Lloyd T.R. Staged reconstruction for hypoplastic left heart syndrome. Contemporary results. // Ann Surg. 1996. Vol. 224. P. 387-394.
4. Curzon C.L., Milford-Beland S., Li J.S., et al. Cardiac surgery in infants with low birth weight is associated with increased mortality: analysis of the Society of Thoracic Surgeons Congenital Heart Database. // J Thorac Cardiovasc Surg 2008. Vol. 135. P. 546-551.
5. Forbess J.M., Cook N., Roth S.J., Serraf A., Mayer J.E. Jr, Jonas R.A. Ten-year institutional experience with palliative surgery for hypoplastic left heart syndrome. Risk factors related to stage I mortality. // Circulation 1995. Vol. 92 (9 Suppl). P. II262-266.
6. Gelehrter S., Fifer C.G., Armstrong A., Hirsch J., Gajarski R. Outcomes of hypoplastic left heart syndrome in low-birthweight patients. // Pediatr Cardiol 2011. Vol. 32. P. 1175-1181.
7. Hirsch J.C., Gurney J.G., Donohue J.E., Gebremariam A., Bove E.L., Ohye R.G. Hospital mortality for Norwood and arterial switch operations as a function of institutional volume. // Pediatr Cardiol. 2008. Vol. 29. P. 713-717.
8. Kalfa D., Krishnamurthy G., Duchon J., et al. Outcomes of cardiac surgery in patients weighing <2.5 kg: affect of patient-dependent and -independent variables. // J Thorac Cardiovasc Surg 2014. Vol. 148. P. 2499-2506.e1.
9. Kalfa D., Krishnamurthy G., Levasseur S., Najjar M., Chai P., Chen J., Quaegebeur J., Bacha E. Norwood Stage I Palliation in Patients Less Than or Equal to 2.5 kg: Outcomes and Risk Analysis. // Ann Thorac Surg. 2015. Vol. 100 (1). P. 167-173.
10. Ohye R.G., Sleeper L.A., Mahony L., et al. Pediatric Heart Network Investigators. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. // N. Engl. J. Med. 2010. Vol. 362. P. 1980-1992.
11. Pizarro C., Davis D.A., Galantowicz M.E., Munro H., Gidding S.S., Norwood W.I. Stage I palliation for hypoplastic left heart syndrome in low birth weight neonates: can we justify it? // Eur J Cardiothorac Surg. 2002. Vol. 21. P. 716-720.
12. Tweddell J.S., Sleeper L.A., Ohye R.G., et al. Pediatric Heart Network Investigators. Intermediate-term mortality and cardiac transplantation in infants with single-ventricle lesions: risk factors and their interaction with shunt type. J Thorac Cardiovasc Surg 2012;144:152–9. (https://www.jtcvs.org/article/S0022-5223(12)00018-9/abstract)
13. Ugonabo N, Hirsch-Romano J.C., Uzark K. The role of home monitoring in interstage management of infants following the Norwood procedure. // World J Pediatr Congenit Heart Surg. 2015. Vol. 6(2). P. 266-273.
14. Weinstein S., Gaynor J.W., Bridges N.D., et al. Early survival of infants weighing 2.5 kilograms or less undergoing first-stage reconstruction for hypoplastic left heart syndrome. // Circulation. 1999. Vol. 100 (19 Suppl). P. II167–70.
2. Матюшов В.Н., Синельников Ю.С., Сойнов И.А., Корнилов И.А., Прохоров С.Н., Чащин О.В., Иванцов С.М. Анализ опыта хирургического лечения общего артериального ствола. // Бюллетень Сибирского отделения Российской академии медицинских наук. 2014. Т. 34. № 1. С. 60-66.
3. Bove E.L., Lloyd T.R. Staged reconstruction for hypoplastic left heart syndrome. Contemporary results. // Ann Surg. 1996. Vol. 224. P. 387-394.
4. Curzon C.L., Milford-Beland S., Li J.S., et al. Cardiac surgery in infants with low birth weight is associated with increased mortality: analysis of the Society of Thoracic Surgeons Congenital Heart Database. // J Thorac Cardiovasc Surg 2008. Vol. 135. P. 546-551.
5. Forbess J.M., Cook N., Roth S.J., Serraf A., Mayer J.E. Jr, Jonas R.A. Ten-year institutional experience with palliative surgery for hypoplastic left heart syndrome. Risk factors related to stage I mortality. // Circulation 1995. Vol. 92 (9 Suppl). P. II262-266.
6. Gelehrter S., Fifer C.G., Armstrong A., Hirsch J., Gajarski R. Outcomes of hypoplastic left heart syndrome in low-birthweight patients. // Pediatr Cardiol 2011. Vol. 32. P. 1175-1181.
7. Hirsch J.C., Gurney J.G., Donohue J.E., Gebremariam A., Bove E.L., Ohye R.G. Hospital mortality for Norwood and arterial switch operations as a function of institutional volume. // Pediatr Cardiol. 2008. Vol. 29. P. 713-717.
8. Kalfa D., Krishnamurthy G., Duchon J., et al. Outcomes of cardiac surgery in patients weighing <2.5 kg: affect of patient-dependent and -independent variables. // J Thorac Cardiovasc Surg 2014. Vol. 148. P. 2499-2506.e1.
9. Kalfa D., Krishnamurthy G., Levasseur S., Najjar M., Chai P., Chen J., Quaegebeur J., Bacha E. Norwood Stage I Palliation in Patients Less Than or Equal to 2.5 kg: Outcomes and Risk Analysis. // Ann Thorac Surg. 2015. Vol. 100 (1). P. 167-173.
10. Ohye R.G., Sleeper L.A., Mahony L., et al. Pediatric Heart Network Investigators. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. // N. Engl. J. Med. 2010. Vol. 362. P. 1980-1992.
11. Pizarro C., Davis D.A., Galantowicz M.E., Munro H., Gidding S.S., Norwood W.I. Stage I palliation for hypoplastic left heart syndrome in low birth weight neonates: can we justify it? // Eur J Cardiothorac Surg. 2002. Vol. 21. P. 716-720.
12. Tweddell J.S., Sleeper L.A., Ohye R.G., et al. Pediatric Heart Network Investigators. Intermediate-term mortality and cardiac transplantation in infants with single-ventricle lesions: risk factors and their interaction with shunt type. J Thorac Cardiovasc Surg 2012;144:152–9. (https://www.jtcvs.org/article/S0022-5223(12)00018-9/abstract)
13. Ugonabo N, Hirsch-Romano J.C., Uzark K. The role of home monitoring in interstage management of infants following the Norwood procedure. // World J Pediatr Congenit Heart Surg. 2015. Vol. 6(2). P. 266-273.
14. Weinstein S., Gaynor J.W., Bridges N.D., et al. Early survival of infants weighing 2.5 kilograms or less undergoing first-stage reconstruction for hypoplastic left heart syndrome. // Circulation. 1999. Vol. 100 (19 Suppl). P. II167–70.
REFERENCES
1. Voitov A.V., Omelchenko A.Yu., Soinov I.A., Nichay N.R., Gorbatykh A.V., Kulyabin Yu.Yu., Arkhipov A.N., Bogachev-Prokofiev A.V. ., Karaskov A.M. The result of using a venous valve-containing allograft in the rehabilitation of pulmonary arteries in a newborn with atresia of the pulmonary artery and a defect in the interventricular septum: a clinical case. // Patologiya krovoobrashcheniya i kardiokhirurgiya. 2018.Vol. 22. No. 2. P. 68-72. DOI: 10.21688/1681-3472-2018-2-68-72 (in Russian)
2. Matyushov VN, Sinelnikov Yu.S., Soinov I.A., Kornilov I.A., Prokhorov S.N., Chashchin O.V., Ivantsov S.M. Analysis of the experience of surgical treatment of the common arterial trunk. // Byulleten' Sibirskogo otdeleniya Rossiyskoy akademii meditsinskikh nauk. 2014. Vol. 34. No. 1. P. 60-66. (in Russian)
3. Bove E.L., Lloyd T.R. Staged reconstruction for hypoplastic left heart syndrome. Contemporary results. // Ann Surg. 1996. Vol. 224. P. 387-394.
4. Curzon C.L., Milford-Beland S., Li J.S., et al. Cardiac surgery in infants with low birth weight is associated with increased mortality: analysis of the Society of Thoracic Surgeons Congenital Heart Database. // J Thorac Cardiovasc Surg 2008. Vol. 135. P. 546-551.
5. Forbess J.M., Cook N., Roth S.J., Serraf A., Mayer J.E. Jr, Jonas R.A. Ten-year institutional experience with palliative surgery for hypoplastic left heart syndrome. Risk factors related to stage I mortality. // Circulation 1995. Vol. 92 (9 Suppl). P. II262-266.
6. Gelehrter S., Fifer C.G., Armstrong A., Hirsch J., Gajarski R. Outcomes of hypoplastic left heart syndrome in low-birthweight patients. // Pediatr Cardiol 2011. Vol. 32. P. 1175-1181.
7. Hirsch J.C., Gurney J.G., Donohue J.E., Gebremariam A., Bove E.L., Ohye R.G. Hospital mortality for Norwood and arterial switch operations as a function of institutional volume. // Pediatr Cardiol. 2008. Vol. 29. P. 713-717.
8. Kalfa D., Krishnamurthy G., Duchon J., et al. Outcomes of cardiac surgery in patients weighing <2.5 kg: affect of patient-dependent and -independent variables. // J Thorac Cardiovasc Surg 2014. Vol. 148. P. 2499-2506.e1.
9. Kalfa D., Krishnamurthy G., Levasseur S., Najjar M., Chai P., Chen J., Quaegebeur J., Bacha E. Norwood Stage I Palliation in Patients Less Than or Equal to 2.5 kg: Outcomes and Risk Analysis. // Ann Thorac Surg. 2015. Vol. 100 (1). P. 167-173.
10. Ohye R.G., Sleeper L.A., Mahony L., et al. Pediatric Heart Network Investigators. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. // N. Engl. J. Med. 2010. Vol. 362. P. 1980-1992.
11. Pizarro C., Davis D.A., Galantowicz M.E., Munro H., Gidding S.S., Norwood W.I. Stage I palliation for hypoplastic left heart syndrome in low birth weight neonates: can we justify it? // Eur J Cardiothorac Surg. 2002. Vol. 21. P. 716-720.
12. Tweddell J.S., Sleeper L.A., Ohye R.G., et al. Pediatric Heart Network Investigators. Intermediate-term mortality and cardiac transplantation in infants with single-ventricle lesions: risk factors and their interaction with shunt type. J Thorac Cardiovasc Surg 2012;144:152–9. (https://www.jtcvs.org/article/S0022-5223(12)00018-9/abstract)
13. Ugonabo N, Hirsch-Romano J.C., Uzark K. The role of home monitoring in interstage management of infants following the Norwood procedure. // World J Pediatr Congenit Heart Surg. 2015. Vol. 6(2). P. 266-273.
14. Weinstein S., Gaynor J.W., Bridges N.D., et al. Early survival of infants weighing 2.5 kilograms or less undergoing first-stage reconstruction for hypoplastic left heart syndrome. // Circulation. 1999. Vol. 100 (19 Suppl). P. II167–70.
Published
2020-04-09
How to Cite
СОЙНОВ, Илья Александрович et al.
Palliative surgery of a patient with hypoplastic left heart syndrome and low body weight..
Sibirskij Medicinskij Zurnal (Irkutsk) = Siberian Medical Journal (Irkutsk) 16+, [S.l.], v. 158, n. 3, p. 46-49, apr. 2020.
ISSN 1815-7572. Available at: <http://smj.ismu.baikal.ru/index.php/osn/article/view/317>. Date accessed: 01 jan. 2026.
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Section
Clinical cases
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