Norwood procedure

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Norwood procedure
Hypoplastic left heart syndrome.svg
Diagram of a healthy heart and one with Hypoplastic left heart syndrome. In the heart on the right, note the near absence of the left ventricle, which normally provides systemic circulation. Following the three-stage palliation (Norwood, Glenn or hemi-Fontan, then Fontan), blood flow from the right ventricle is rerouted to serve this function, which means that an alternative source of pulmonary circulation must be provided.
ICD-9-CM 35.8

The Norwood procedure is the first of three surgeries intended to create a new functional systemic circuit in patients with hypoplastic left heart syndrome (HLHS) and other complex heart defects with single ventricle physiology. [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]

Contents

Variations of Norwood procedure, or Stage 1 palliation, have been proposed and adopted over the last 30 years; however, the key steps have remain unchanged. In order to utilize the right ventricle as the main blood pumping mechanism into the systemic and pulmonary circulation, a connection between left and right atria is established via atrial septectomy. Next a connection between the right ventricle and aorta is forged with the reconstruction of the narrowed outflow track using a tissue graft from the distal main pulmonary artery. [4] Lastly, an aortopulmonary shunt is created connecting the aorta to the main pulmonary artery to provide pulmonary blood flow.

The second surgery, also known as the Glenn procedure, focuses on separating the systemic and pulmonary circulation once pulmonary vascular resistance drops. This is accomplished by removing the aortopulmonary conduit followed by the creation of a bidirectional SVC-pulmonary shunt. [5]

The third surgery (Stage 3) is the Fontan procedure, in which the inferior vena cava (IVC, the large vein carrying blood back to the heart from the lower part of the body) is connected to the branch pulmonary arteries. After this surgery is completed, all the venous blood returning from the body flows directly to the lungs. [6]

Indications

Norwood procedure Norwood Surgical Correction.png
Norwood procedure

Norwood procedure is most commonly performed to treat hypoplastic left heart syndrome, however variations of this procedure are also used for palliation of mitral and tricuspid atresia, [7] and subsets of transposition of great arteries (TGA). [8]

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. [9]

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. [10]

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. [11]

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.[ citation needed ]

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. [12]

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. [13]

Interstage Sequelae and Long Term Outcomes

Infants normally undergo the Norwood procedure within the first three to seven days postpartum. The decision for earlier intervention rests in the neonate's cardiopulmonary status. Interstage period, or the period after Norwood procedure and before stage II palliation remains associated with mortality rates ranging from 2%-20%. [14] A few risk factors contributing to increased interstage mortality have been identified: gestational age less than 37 weeks at delivery, Hispanic ethnicity, and census block poverty level. Airway complications, as well as difficulties with feeding, and upper respiratory infections have also been associated with increased mortality. Despite results from SVR trial, Blalock-Taussig conduits remained significantly associated with interim death compared to RVPA bypass up to 1 year post palliation. [15]

Immediate post surgical complications have been reported by multiple studies to involve paralysis of the vocal cords 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. [16]

With the increasing survival rates even for the most complicated congenital heart diseases such as HLHS, long term outcomes on neurodevelopemental have been closely analyzed after the SVR trial. Data from et al. Ohye demonstrated below average performance as scored using Psychomotor- Developmental Index (PDI) and Mental Developmental Index (MDI) of the Bayley Scales of Infant Development in infants 14 months and older. Additionally, Ages and Stages Questionnaire (ASQ) was used for the three year follow up. Consisting of five domains the ASQ evaluates Communication, Gross Motor, Fine Motor, Problem solving, and Personal/Social interactions. Again statistical analysis demonstrated below average performance for Norwood patients compared to reference population in all five of the aforementioned domains. Risk factors directly affecting PDI score were found to be lower birth weight, longer length of stay following Norwood procedure, and more complications between Norwood procedure and 1 year of age. [17]

Prenatal detection of a HLHS defect is critical to successful palliation. As HLHS is incompatible with life postpartum, the only treatment is surgery. Despite surgical intervention, infants are at a constant risk of circulatory failure and death. In fact recent reviews suggest that only 66% of infants will live past 5 years of age. Furthermore, infants have a mortality risk of 1% per year following last stage palliation. [18]

History

First ever series of documented Norwood procedures were performed by Dr. William Imon Norwood between 1979 and 1981. [19] Dr. Norwood was an American physician who completed his fellowship in cardiothoracic pediatric surgery at Boston Children's Medical Center (BCMC), Boston Massachusetts. [20] 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. [21]

Related Research Articles

dextro-Transposition of the great arteries Medical condition

dextro-Transposition of the great arteries is a potentially life-threatening birth defect in the large arteries of the heart. The primary arteries are transposed.

A cyanotic heart defect is any congenital heart defect (CHD) that occurs due to deoxygenated blood bypassing the lungs and entering the systemic circulation, or a mixture of oxygenated and unoxygenated blood entering the systemic circulation. It is caused by structural defects of the heart such as right-to-left or bidirectional shunting, malposition of the great arteries, or any condition which increases pulmonary vascular resistance. The result may be the development of collateral circulation.

<span class="mw-page-title-main">Fontan procedure</span> Surgical procedure used in children with univentricular hearts

The Fontan procedure or Fontan–Kreutzer procedure is a palliative surgical procedure used in children with univentricular hearts. It involves diverting the venous blood from the inferior vena cava (IVC) and superior vena cava (SVC) to the pulmonary arteries. The procedure varies for differing congenital heart pathologies. For example in tricuspid atresia, the procedure can be done where the blood does not pass through the morphologic right ventricle; i.e., the systemic and pulmonary circulations are placed in series with the functional single ventricle. Whereas in hypoplastic left heart syndrome, the heart is more reliant on the more functional right ventricle to provide blood flow to the systemic circulation. The procedure was initially performed in 1968 by Francis Fontan and Eugene Baudet from Bordeaux, France, published in 1971, simultaneously described in 1971 by Guillermo Kreutzer from Buenos Aires, Argentina, and finally published in 1973.

<span class="mw-page-title-main">Blalock–Thomas–Taussig shunt</span> Cardiac surgery procedure

The Blalock–Thomas–Taussig shunt, previously known as the Blalock-Taussig Shunt, is a surgical procedure used to increase blood flow to the lungs in some forms of congenital heart disease such as pulmonary atresia and tetralogy of Fallot and are common causes of blue baby syndrome. The procedure involves connecting a branch of the subclavian artery or carotid artery to the pulmonary artery. In modern practice, this procedure is temporarily used to direct blood flow to the lungs and relieve cyanosis while the infant is waiting for corrective or definitive surgery when their heart is larger. The BTT shunt is used in the first step of the three-stage palliation.

The Rastelli procedure is an open heart surgical procedure developed by Italian physician and cardiac surgery researcher, Giancarlo Rastelli, in 1967 at the Mayo Clinic, and involves using a pulmonary or aortic homograft conduit to relieve pulmonary obstruction in double outlet right ventricle with pulmonary stenosis.

<span class="mw-page-title-main">Hypoplastic left heart syndrome</span> Type of congenital heart defect

Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped and incapable of supporting the systemic circulation. It is estimated to account for 2-3% of all congenital heart disease. Early signs and symptoms include poor feeding, cyanosis, and diminished pulse in the extremities. The etiology is believed to be multifactorial resulting from a combination of genetic mutations and defects resulting in altered blood flow in the heart. Several structures can be affected including the left ventricle, aorta, aortic valve, or mitral valve all resulting in decreased systemic blood flow.

<span class="mw-page-title-main">Transposition of the great vessels</span> Group of congenital heart defects

Transposition of the great vessels (TGV) is a group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels: superior and/or inferior venae cavae, pulmonary artery, pulmonary veins, and aorta. Congenital heart diseases involving only the primary arteries belong to a sub-group called transposition of the great arteries (TGA), which is considered the most common congenital heart lesion that presents in neonates.

<span class="mw-page-title-main">Pulmonary atresia</span> Medical condition

Pulmonary atresia is a congenital malformation of the pulmonary valve in which the valve orifice fails to develop. The valve is completely closed thereby obstructing the outflow of blood from the heart to the lungs. The pulmonary valve is located on the right side of the heart between the right ventricle and pulmonary artery. In a normal functioning heart, the opening to the pulmonary valve has three flaps that open and close.

<span class="mw-page-title-main">Tricuspid atresia</span> Medical condition

Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic (undersized) or absent right ventricle. This defect is contracted during prenatal development, when the heart does not finish developing. It causes the systemic circulation to be filled with relatively deoxygenated blood. The causes of tricuspid atresia are unknown.

<span class="mw-page-title-main">Double inlet left ventricle</span> Medical condition

A double inlet left ventricle (DILV) or "single ventricle", is a congenital heart defect appearing in 5 in 100,000 newborns, where both the left atrium and the right atrium feed into the left ventricle. The right ventricle is hypoplastic or does not exist.

<span class="mw-page-title-main">Bidirectional Glenn procedure</span>

The bidirectional Glenn (BDG) shunt, or bidirectional cavopulmonary anastomosis, is a surgical technique used in pediatric cardiac surgery procedure used to temporarily improve blood oxygenation for patients with a congenital cardiac defect resulting in a single functional ventricle. Creation of a bidirectional shunt reduces the amount of blood volume that the heart needs to pump at the time of surgical repair with the Fontan procedure.

<span class="mw-page-title-main">Hypoplastic right heart syndrome</span> Type of congenital heart disease

Hypoplastic right heart syndrome or HRHS is a congenital heart defect in which the structures on the right side of the heart, particularly the right ventricle, are underdeveloped. This defect causes inadequate blood flow to the lungs, and thus a cyanotic infant.

<span class="mw-page-title-main">Anomalous left coronary artery from the pulmonary artery</span> Medical condition

Anomalous left coronary artery from the pulmonary artery is a rare congenital anomaly occurring in approximately 1 in 300,000 liveborn children. The diagnosis comprises between 0.24 and 0.46% of all cases of congenital heart disease. The anomalous left coronary artery (LCA) usually arises from the pulmonary artery instead of the aortic sinus. In fetal life, the high pressure in the pulmonic artery and the fetal shunts enable oxygen-rich blood to flow in the LCA. By the time of birth, the pressure will decrease in the pulmonic artery and the child will have a postnatal circulation. The myocardium which is supplied by the LCA, will therefore be dependent on collateral blood flow from the other coronary arteries, mainly the RCA. Because the pressure in RCA exceeds the pressure in LCA a collateral circulation will increase. This situation ultimately can lead to blood flowing from the RCA into the LCA retrograde and into the pulmonary artery, thus forming a left-to-right shunt.

<span class="mw-page-title-main">Sano shunt</span>

A Sano shunt is a shunt from the right ventricle to the pulmonary circulation.

The Senning procedure is an atrial switch heart operation performed to treat transposition of the great arteries. It is named after its inventor, the Swedish cardiac surgeon Åke Senning (1915–2000), also known for implanting the first permanent cardiac pacemaker in 1958.

Fetal aortic stenosis is a disorder that occurs when the fetus’ aortic valve does not fully open during development. The aortic valve is a one way valve that is located between the left ventricle and the aorta, keeping blood from leaking back into the ventricle. It has three leaflets that separate when the ventricle contracts to allow blood to move from the ventricle to the aorta. These leaflets come together when the ventricle relaxes.

The Damus–Kaye–Stansel (DKS) procedure is a cardiovascular surgical procedure used as part of the repair of some congenital heart defects. This procedure joins the pulmonary artery and the aorta in situations where the systemic circulation is obstructed. It is commonly used when a patient has the combination of a small left ventricle and a transposition of the great arteries (TGA); in this case, the procedure allows blood to flow from the left ventricle to the aorta.

The LeCompte maneuver is a technique used in open heart surgery, primarily on infants and children. The maneuver entails cutting the main pulmonary artery and moving it anterior to the aorta before reattaching the pulmonary artery during the following reconstruction of the great vessels. It allows the surgeon to reconstruct the right ventricular outflow tract without needing to connect the proximal and distal sections with a graft. It also enables the surgeon to avoid compressing the coronary arteries and relieves compression of the bronchi in cases where the pulmonary artery is severely dilated or aneurysmal. If both pulmonary arteries are not mobilized adequately, they can become stretched, leading to pulmonic stenosis.

The Yasui procedure is a pediatric heart operation used to bypass the left ventricular outflow tract (LVOT) that combines the aortic repair of the Norwood procedure and a shunt similar to that used in the Rastelli procedure in a single operation. It is used to repair defects that result in the physiology of hypoplastic left heart syndrome even though both ventricles are functioning normally. These defects are common in DiGeorge syndrome and include interrupted aortic arch and LVOT obstruction (IAA/LVOTO); aortic atresia-severe stenosis with ventricular septal defect (AA/VSD); and aortic atresia with interrupted aortic arch and aortopulmonary window. This procedure allows the surgeon to keep the left ventricle connected to the systemic circulation while using the pulmonary valve as its outflow valve, by connecting them through the ventricular septal defect. The Yasui procedure includes a modified Damus–Kaye–Stansel procedure to connect the aortic and pulmonary roots, allowing the coronary arteries to remain perfused. It was first described in 1987.

Single ventricle is a rare congenital heart defect, which constitutes just over 1% of congenital cardiovascular diseases. The single functional ventricle could be morphologically right or left with the second ventricle usually hypoplastic and/or insufficiently functional. Therefore, there are several subtypes of the disease, depending on which ventricle is underdeveloped.

References

  1. ↑ "Norwood Procedure | Hypoplastic Left Heart Syndrome | Children's Wisconsin". childrenswi.org. Retrieved 2021-11-15.
  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. doi: 10.1016/s0022-5223(19)39288-8 . PMID   6168869.
  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. doi:10.1056/NEJM198301063080106. PMID   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. doi:10.1038/pr.2016.194. ISSN   0031-3998. PMC   5313512 . PMID   27701379.
  5. ↑ Gregory's pediatric anesthesia (5th ed.). Chichester, West Sussex: Wiley-Blackwell. 2012. p. 622. ISBN   978-1-4443-3346-6.
  6. ↑ Davies, Ryan R.; Chen, Jonathan M.; Mosca, Ralph S. (2011-01-01). "The Fontan Procedure: Evolution in Technique; Attendant Imperfections and Transplantation for "Failure"". Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual. 14 (1): 55–66. doi:10.1053/j.pcsu.2011.01.014. ISSN   1092-9126. PMID   21444050.
  7. ↑ 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. doi: 10.1016/j.ejcts.2008.09.016 . ISSN   1010-7940. PMID   18996714.
  8. ↑ 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. doi: 10.1016/j.ejcts.2008.09.016 . ISSN   1873-734X. PMID   18996714.
  9. ↑ 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. 98 (1): 142–150. doi: 10.1016/j.athoracsur.2014.02.078 . ISSN   0003-4975. PMID   24793687.
  10. ↑ Corno A, Festa GP (8 December 2008). Congenital Heart Defects. Decision Making for Surgery: CT-Scan and Clinical Correlations. Springer. pp. 123–. ISBN   978-3-7985-1718-9 . Retrieved 24 June 2011.
  11. ↑ Barron, David J. (2013-01-01). "The Norwood Procedure: In favor of the RV-PA Conduit". Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual. Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual 2013. 16 (1): 52–58. doi:10.1053/j.pcsu.2013.01.002. ISSN   1092-9126. PMID   23561818.
  12. ↑ 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. doi:10.1161/CIRCULATIONAHA.116.022816. ISSN   1524-4539. PMC   5119545 . PMID   27777296.
  13. ↑ Michielon, Guido; DiSalvo, Giovanni; Fraisse, Alain; Carvalho, Julene S; Krupickova, Sylvia; Slavik, Zdenek; Bartsota, Margarita; Daubeney, Pierce; Bautista, Carles; Desai, Ajay; Burmester, Margarita (2020-06-01). "In-hospital interstage improves interstage survival after the Norwood stage 1 operation". European Journal of Cardio-Thoracic Surgery. 57 (6): 1113–1121. doi: 10.1093/ejcts/ezaa074 . ISSN   1010-7940. PMID   32236554.
  14. ↑ 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. doi: 10.1016/j.jtcvs.2009.07.063 . ISSN   1097-685X. PMID   19879598.
  15. ↑ Ghanayem NS, Allen KR, Tabbutt S, Atz AM, Clabby ML, Cooper DS, et al. (October 2012). "Interstage mortality after the Norwood procedure: Results of the multicenter Single Ventricle Reconstruction trial". The Journal of Thoracic and Cardiovascular Surgery. 144 (4): 896–906. doi:10.1016/j.jtcvs.2012.05.020. PMC   3985484 . PMID   22795436.
  16. ↑ 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. doi: 10.1017/S104795111800135X . ISSN   1467-1107. PMID   30223915. S2CID   52290933.
  17. ↑ Ohye RG, Schranz D, D'Udekem Y (October 2016). "Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions". Circulation. 134 (17): 1265–1279. doi:10.1161/CIRCULATIONAHA.116.022816. PMC   5119545 . PMID   27777296.
  18. ↑ Roeleveld, Peter P.; Axelrod, David M.; Klugman, Darren; Jones, Melissa B.; Chanani, Nikhil K.; Rossano, Joseph W.; Costello, John M. (November 2018). "Hypoplastic left heart syndrome: from fetus to fontan". Cardiology in the Young. 28 (11): 1275–1288. doi: 10.1017/S104795111800135X . ISSN   1467-1107. PMID   30223915. S2CID   52290933.
  19. ↑ Norwood, William I.; Lang, Peter; Castaneda, Aldo R.; Campbell, David N. (1981-10-01). "Experience with operations for hypoplastic left heart syndrome". The Journal of Thoracic and Cardiovascular Surgery. 82 (4): 511–519. doi: 10.1016/S0022-5223(19)39288-8 . ISSN   0022-5223. PMID   6168869.
  20. ↑ "Dr. William Norwood Jr. Obituary (1941 - 2020) Albuquerque Journal". Legacy.com. Retrieved 2021-11-17.
  21. ↑ Skalski, Janusz H. (December 2020). "William Imon Norwood, 1941–2020". Kardiochirurgia I Torakochirurgia Polska = Polish Journal of Cardio-Thoracic Surgery. 17 (4): 214–216. doi:10.5114/kitp.2020.102638. ISSN   1731-5530. PMC   7848620 . PMID   33552190.
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