Norwood procedure explained

Norwood procedure

The Norwood procedure is the first of three palliative surgeries for patients with hypoplastic left heart syndrome (HLHS) and other complex heart defects with single ventricle physiology intended to create a new functional single ventricle system.[1] The first successful Norwood procedure involving the use of a cardiopulmonary bypass was reported by Dr. William Imon Norwood, Jr. and colleagues in 1981.[2] [3]

Variations of the Norwood procedure, or Stage 1 palliation, have been proposed and adopted over the last 30 years; however, its basic components have remained unchanged. The purpose of the procedure is to utilize the right ventricle as the main chamber pumping blood to the body and lungs. A connection between left and right atria (collecting chambers of the heart) is established via atrial septectomy, allowing blood arriving from the lungs to travel to the right ventricle. Next a connection between the right ventricle and aorta is created using a tissue graft from the main pulmonary artery.[4] Lastly, an aortopulmonary shunt is created to provide blood flow to the lungs from the systemic circulation. The most common shunts are the Modified Blalock Taussig shunt (MBTS) or right ventricle- to pulmonary artery shunt (RVPA or Sano shunt).

Most patients who undergo a Norwood procedure will proceed to further stages of single ventricle palliation. A second surgery, also known as the Glenn procedure, occurs at 4-6 months of age. The third surgery is the Fontan procedure, occurring when patients are 3-5 years of age.

Indications

Norwood procedure is most commonly performed to treat hypoplastic left heart syndrome, double outlet right ventricle, double inlet left ventricle, and other single ventricle congenital heart defects. Variations are also used for palliation of mitral and tricuspid atresia[5] and subsets of transposition of great arteries (TGA).[6]

Without surgical repair, infants born with a single ventricle cardiac defect face almost certain mortality in the first year of life.[7] [8] [9] In these conditions, the most urgent problem is that the heart is unable to pump blood to the systemic circulation (i.e. to the body). The goal of these three surgeries is to ultimately connect the single ventricle to the systemic circulation. To accomplish this, blood flow to the lungs is disrupted, and therefore an alternative path must be created to provide blood flow to the lungs.[10]

Contraindications

There are numerous factors that increase the risk of the Norwood procedure and are relative contraindications. Those factors include Low birth weight, extremely premature delivery, poor ventricular function, Intraventricular hemorrhage, severe non-cardiac congenital defects, and genetic syndromes with poor prognosis.

Alternate Options

While the Norwood procedure is the standard of care for single ventricle cardiac defects, there are other treatment options for patients depending on their unique anatomy. One option is the Hybrid procedure which is done via cardiac catheterization and surgery.[11] [12] A stent is placed in the ductus arteriosus to keep it patent and bands are placed over both the left and right pulmonary arteries to limit pressure and over-circulation to the lungs.[13] Another option is cardiac transplantation, although this is uncommon due to the limited availability of neonatal donor hearts.[14] [15] Families can also elect to pursue comfort care for their newborns, especially if there are concomitant anatomic defects or genetic syndromes with poor prognosis.[16]

Process

Entry to the body cavity for the Norwood procedure is gained by a vertical incision above the sternum. Separation of the sternum is necessary. This surgery is complex and may vary slightly depending on the diagnosis and overall condition of the heart. The surgery on the heart can be divided into two main steps.[17]

Providing systemic circulation

The main pulmonary artery is separated from the left and right portions of the pulmonary artery and joined with the upper portion of the aorta. Widening of the pulmonary artery is often necessary, and may be accomplished by using the patient's existing biological tissue, or appropriate animal tissue. This allows the blood, a mixture of oxygenated and deoxygenated, to be pumped to the body via the morphologic right ventricle, through the pulmonary valve. At this point in the surgery, the right ventricle is directly connected to systemic circulation through the Neoaorta or the reconstructed aortic outflow track. Second step of the procedure establishes blood flow to the lungs.[18]

Providing pulmonary circulation

Variations to this step have been proposed over the years, however only two have been adapted in general practice over the last 20 years. In both cases a conduit is used to direct blood flow into the lungs, however anatomic anchoring varies. There are two different types of shunts used during the procedure: Modified Blalock Taussig or (MBTS) and right ventricle- to pulmonary artery shunt (RVPA or Sano shunt). MBTS shunt provides connection from the pulmonary artery to brachiocephalic artery or subclavian artery, while the RVPA conduit provides connection from right ventricle to pulmonary artery.

The Single Ventricle Reconstruction conducted in 2005 compared the two conduits at one, three and five year intervals. Although RVPA shunts performed better at the one and three year end points, five year follow up demonstrated no difference between survival or improvement in freedom from transplantation.[19]

After Norwood procedure infants enter the interstage which typically lasts up to 5 months. During this period the patients are medically optimized using diuretics and vasodilators.[20]

Outcomes

The Norwood procedure is a complex and high-risk surgery with high rates of morbidity and mortality despite advancements in surgical technique, perioperative care, and postoperative monitoring.[21]

Surgical Complications

Immediate post surgical complications have been reported by multiple studies to involve hemorrhage, vocal cord paralysis due to close proximity of the recurrent laryngeal nerve to the cardiac sack, cardiac arrhythmias as a result of potential cardiac tissue manipulation and damage, and protein-losing enteropathy.[22] Other surgical complications include low cardiac output syndrome, atrioventricular valve regurgitation, aortic valve insufficiency, ventricular dysfunction, seizures, stroke, shunt thrombosis, infection, cardiac arrest, and death.[23]

Interstage Period

The interstage period is the period after the Norwood procedure and before stage II pallation (Glenn procedure, typically 4-6 months of age). This time is very high-risk for infants because the single ventricle must pump to both the systemic and pulmonary circulations, with mortality rates ranging from 2%-20%.[24]

Due to the balance required to maintain adequate blood flow to the systemic and pulmonary circulations, infants in the interstage period face multiple risks:

Long-Term Outcomes

Long-term survival rates for children with single ventricle physiology are improving as medical and surgical advancements continue. In the major SVR study (Single Ventricle Reconstruction) the transplant-free survival rate was only 54-59% amongst patients who underwent the Norwood procedure.[28] These patients experience ongoing health challenges and require lifelong cardiology follow-up.

Neurodevelopment

Children with single-ventricle physiology who undergo the Norwood procedure often experience neurodevelopmental impairment.[29] Neurodevelopmental and behavioral impairments are the most common long-term morbidity for children with single ventricle cardiac defects.[30] Children's hospitals have begun to implement multidisciplinary neurodevelopmental care teams as part of the standard of care for this population.[31] The impact of these interventional programs remains an active area of research.

Factors affecting neurodevelopment in these children include:[32]

Children who undergo the Norwood procedure may experience a range of neurodevelopmental issues, such as:

History

First ever series of documented Norwood procedures were performed by Dr. William Imon Norwood between 1979 and 1981.[37] Dr. Norwood was an American physician who completed his fellowship in cardiothoracic pediatric surgery at Boston Children's Medical Center (BCMC), Boston Massachusetts.[38] During his time at BCMC he became interested in the most complex congenital heart defects, particularly HLHS. Under direct supervision of his program mentor Dr. Aldo Castanedo, he performed and later perfected what would become the three stage Norwood palliation. After successful publication of his work in 1981, Dr. Norwood joined the Project Hope stationed in Krakow, Poland. There, he continued to develop and refine his work: he was responsible for Poland's first ever Fontan procedure in a patient with single ventricle pathology.[39]

Notes and References

  1. Web site: Norwood Procedure Hypoplastic Left Heart Syndrome Children's Wisconsin . 2021-11-15 . childrenswi.org . en.
  2. Norwood WI, Lang P, Casteneda AR, Campbell DN . October 1981 . Experience with operations for hypoplastic left heart syndrome . The Journal of Thoracic and Cardiovascular Surgery . 82 . 4 . 511–9 . 10.1016/s0022-5223(19)39288-8 . 6168869 . free.
  3. Norwood WI, Lang P, Hansen DD . January 1983 . Physiologic repair of aortic atresia-hypoplastic left heart syndrome . The New England Journal of Medicine . 308 . 1 . 23–6 . 10.1056/NEJM198301063080106 . 6847920.
  4. Yabrodi . Mouhammad . Mastropietro . Christopher W. . January 2017 . Hypoplastic left heart syndrome: from comfort care to long-term survival . Pediatric Research . 81 . 1–2 . 142–149 . 10.1038/pr.2016.194 . 0031-3998 . 5313512 . 27701379.
  5. Lotto . Attilio A. . Hosein . Riad . Jones . Timothy J. . Barron . David J. . Brawn . William J. . 2009-01-01 . Outcome of the Norwood procedure in the setting of transposition of the great arteries and functional single left ventricle . European Journal of Cardio-Thoracic Surgery . 35 . 1 . 149–155 . 10.1016/j.ejcts.2008.09.016 . 1010-7940 . 18996714 . free.
  6. Lotto . Attilio A. . Hosein . Riad . Jones . Timothy J. . Barron . David J. . Brawn . William J. . January 2009 . Outcome of the Norwood procedure in the setting of transposition of the great arteries and functional single left ventricle . European Journal of Cardio-Thoracic Surgery . 35 . 1 . 149–155; discussion 155 . 10.1016/j.ejcts.2008.09.016 . 1873-734X . 18996714 . free.
  7. Dave . Hitendu . Rosser . Barbara . Knirsch . Walter . Hübler . Michael . Prêtre . René . Kretschmar . Oliver . July 2014 . Hybrid approach for hypoplastic left heart syndrome and its variants: the fate of the pulmonary arteries . European Journal of Cardio-Thoracic Surgery: Official Journal of the European Association for Cardio-Thoracic Surgery . 46 . 1 . 14–19 . 10.1093/ejcts/ezt604 . 1873-734X . 24420367.
  8. Carvajal . Horacio G. . Canter . Matthew W. . Wan . Fei . Eghtesady . Pirooz . November 2023 . Hypoplastic Left Heart Syndrome With Low Birth Weight or Prematurity: What Is the Optimal Approach? . The Annals of Thoracic Surgery . 116 . 5 . 988–995 . 10.1016/j.athoracsur.2023.06.025 . 1552-6259 . 37429513.
  9. Lee . Madonna E. . Kopf . Gary S. . Geirsson . Arnar . Gruber . Peter J. . September 2022 . Pioneers in congenital cardiac surgery: Dr. William Imon Norwood, Jr, MD, PhD . Journal of Cardiac Surgery . 37 . 9 . 2521–2523 . 10.1111/jocs.16694 . 1540-8191 . 35748274.
  10. Sharma . Vikas . Deo . Salil V. . Huebner . Marianne . Dearani . Joseph A. . Burkhart . Harold M. . 2014-07-01 . In Search of the Ideal Pulmonary Blood Source for the Norwood Procedure: A Meta-Analysis and Systematic Review . The Annals of Thoracic Surgery . en . 98 . 1 . 142–150 . 10.1016/j.athoracsur.2014.02.078 . 0003-4975 . 24793687 . free.
  11. Murphy . Michael O. . Bellsham-Revell . Hannah . Morgan . Gareth J. . Krasemann . Thomas . Rosenthal . Eric . Qureshi . Shakeel A. . Salih . Caner . Austin . Conal B. . Anderson . David R. . December 2015 . Hybrid Procedure for Neonates With Hypoplastic Left Heart Syndrome at High-Risk for Norwood: Midterm Outcomes . The Annals of Thoracic Surgery . 100 . 6 . 2286–2290; discussion 2291–2292 . 10.1016/j.athoracsur.2015.06.098 . 1552-6259 . 26433522.
  12. Feinstein . Jeffrey A. . Benson . D. Woodrow . Dubin . Anne M. . Cohen . Meryl S. . Maxey . Dawn M. . Mahle . William T. . Pahl . Elfriede . Villafañe . Juan . Bhatt . Ami B. . Peng . Lynn F. . Johnson . Beth Ann . Marsden . Alison L. . Daniels . Curt J. . Rudd . Nancy A. . Caldarone . Christopher A. . 2012-01-03 . Hypoplastic left heart syndrome: current considerations and expectations . Journal of the American College of Cardiology . 59 . 1 Suppl . S1–42 . 10.1016/j.jacc.2011.09.022 . 1558-3597 . 6110391 . 22192720.
  13. Wilder . Travis J. . Caldarone . Christopher A. . December 2020 . Apples to oranges: Making sense of hybrid palliation for hypoplastic left heart syndrome . JTCVS Open . 4 . 47–54 . 10.1016/j.xjon.2020.10.002 . 2666-2736 . 9390685 . 36004289.
  14. Rai . Vivek . Gładki . Marcin . Dudyńska . Mirosława . Skalski . Janusz . April 2019 . Hypoplastic left heart syndrome [HLHS]: treatment options in present era . Indian Journal of Thoracic and Cardiovascular Surgery . 35 . 2 . 196–202 . 10.1007/s12055-018-0742-z . 0973-7723 . 7525540 . 33061005.
  15. Cleveland . David . Adam Banks . C. . Hara . Hidetaka . Carlo . Waldemar F. . Mauchley . David C. . Cooper . David K. C. . February 2019 . The Case for Cardiac Xenotransplantation in Neonates: Is Now the Time to Reconsider Xenotransplantation for Hypoplastic Left Heart Syndrome? . Pediatric Cardiology . 40 . 2 . 437–444 . 10.1007/s00246-018-1998-1 . 1432-1971 . 30302505.
  16. Iskander . Christopher . Nwankwo . Ugonna . Kumanan . Krithika K. . Chiwane . Saurabh . Exil . Vernat . Lowrie . Lia . Tan . Corinne . Huddleston . Charles . Agarwal . Hemant S. . 2024-07-20 . Comparison of Morbidity and Mortality Outcomes between Hybrid Palliation and Norwood Palliation Procedures for Hypoplastic Left Heart Syndrome: Meta-Analysis and Systematic Review . Journal of Clinical Medicine . 13 . 14 . 4244 . 10.3390/jcm13144244 . free . 2077-0383 . 11277754 . 39064284.
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  19. Ohye. Richard G.. Schranz. Dietmar. D'Udekem. Yves. 2016-10-25. Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions. Circulation. 134. 17. 1265–1279. 10.1161/CIRCULATIONAHA.116.022816. 1524-4539. 5119545. 27777296.
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  21. Kaplinski . Michelle . Ittenbach . Richard F. . Hunt . Mallory L. . Stephan . Donna . Natarajan . Shobha S. . Ravishankar . Chitra . Giglia . Therese M. . Rychik . Jack . Rome . Jonathan J. . Mahle . Marlene . Kennedy . Andrea T. . Steven . James M. . Fuller . Stephanie M. . Nicolson . Susan C. . Spray . Thomas L. . 2020-10-20 . Decreasing Interstage Mortality After the Norwood Procedure: A 30-Year Experience . Journal of the American Heart Association . 9 . 19 . e016889 . 10.1161/JAHA.120.016889 . 2047-9980 . 7792374 . 32964778.
  22. Roeleveld . Peter P. . Axelrod . David M. . Klugman . Darren . Jones . Melissa B. . Chanani . Nikhil K. . Rossano . Joseph W. . Costello . John M. . 2018 . Hypoplastic left heart syndrome: from fetus to fontan . Cardiology in the Young . 28 . 11 . 1275–1288 . 10.1017/S104795111800135X . 1467-1107 . 30223915 . 52290933 . free.
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  24. Furck . Anke Katharina . Uebing . Anselm . Hansen . Jan Hinnerk . Scheewe . Jens . Jung . Olaf . Fischer . Gunther . Rickers . Carsten . Holland-Letz . Tim . Kramer . Hans-Heiner . February 2010 . Outcome of the Norwood operation in patients with hypoplastic left heart syndrome: a 12-year single-center survey . The Journal of Thoracic and Cardiovascular Surgery . 139 . 2 . 359–365 . 10.1016/j.jtcvs.2009.07.063 . 1097-685X . 19879598 . free.
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  27. Cain . Nicole . Saul . J. Philip . Gongwer . Russell . Trachtenberg . Felicia . Czosek . Richard J. . Kim . Jeffrey J. . Kaltman . Jonathon R. . LaPage . Martin J. . Janson . Christopher M. . Singh . Anoop K. . Hill . Allison C. . Landstrom . Andrew P. . Thacker . Deepika . Niu . Mary C. . DeWitt . Elizabeth S. . 2022-04-15 . Relation of Norwood Shunt Type and Frequency of Arrhythmias at 6 Years (from the Single Ventricle Reconstruction Trial) . The American Journal of Cardiology . 169 . 107–112 . 10.1016/j.amjcard.2021.12.056 . 1879-1913 . 35101270.
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