scholarly journals Geometry of the pulmonary arteries before the Fontan operation: can we influence it during the Norwood procedure?

2020 ◽  
Vol 57 (6) ◽  
pp. 1098-1104
Author(s):  
Katarzyna Januszewska ◽  
Pawel Nawrocki ◽  
Anja Lehner ◽  
Julia Stegger ◽  
Felix Kleinerueschkamp ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Fontan Operation ◽  
Pulmonary Arteries ◽  
Norwood Procedure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Second Stage ◽  
The Right ◽  
Left Heart Syndrome ◽  
The Impact

Abstract OBJECTIVES The right ventricle-to-pulmonary artery (RV-PA) shunt provides stable haemodynamics after the Norwood procedure but can influence development of the central pulmonary arteries (PAs). The goal of this study was to analyse the geometry of the central PAs in children with hypoplastic left heart syndrome before the Fontan operation with respect to the RV-PA shunt site and the type of the second-stage operation. METHODS A total of 161 children with hypoplastic left heart syndrome, median age 2.7 (range 1.3–9.8) years and median weight 12.7 (range 7.6–26.1) kg, underwent the Fontan operation after having had the Norwood procedure with an RV-PA shunt. The patients were divided into 2 groups: left-sided RV-PA (L-RV-PA) (n = 129) with the shunt on the left and right-sided RV-PA (n = 32) with the shunt on the right side of the neoaorta. Angiographic data obtained before the Fontan and all cardiac catheterization interventions were analysed retrospectively. RESULTS Between the second and third stages, as well as directly before the Fontan operation, the L-RV-PA group required more PA catheter interventions (P = 0.001 and P = 0.03). In this group, the minimal left PA diameter was smaller than that in the R-RV-PA group (P = 0.021). Leaving the shunt open until the Fontan operation increased the rate of PA interventions in the L-RV-PA group (P = 0.001), but there is no evidence of the impact on the development of the left PAs (P = 0.075). There is also no evidence that the type of the second-stage procedure influences the intervention rate before the Fontan procedure (P = 0.14). CONCLUSIONS Children who have the L-RV-PA shunt require more PA catheter interventions. The right-sided RV-PA shunt and the subsequent Glenn anastomosis in the place of the shunt are associated with distortion-free and more symmetrical development of the central PAs.

Impact of placing a conduit from the right ventricle to the pulmonary arteries as the first stage of further palliation in the Norwood sequence for hypoplasia of the left heart

2007 ◽  
Vol 17 (5) ◽  
pp. 517-522 ◽  
Author(s):  
Martin Kostolny ◽  
Juergen Hoerer ◽  
Andreas Eicken ◽  
Claudia Dietrich ◽  
Christian F. Schreiber ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Hypoplastic Left Heart Syndrome ◽  
Pulmonary Arteries ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Second Stage ◽  
Extracardiac Conduit ◽  
Cavopulmonary Anastomosis ◽  
The Right ◽  
Left Heart Syndrome

AbstractObjectiveWe describe the experience from a single institution with the Norwood sequence of palliation for hypoplasia of the left heart, emphasizing complications related to placement of a conduit from the right ventricle to the pulmonary arteries and their management.MethodsBetween November, 2002 and January, 2006, we palliated 32 patients with hypoplastic left heart syndrome or its variants by placing a conduit from the right ventricle to the pulmonary arteries. We reviewed retrospectively the charts and angiograms from these patients.ResultsHospital survival after construction of the conduit was 90.6%. There were 3 interstage deaths, of which 2 were likely due severe obstruction of the conduit. Stents were implanted into the proximal or medial portions of the conduits of 3 patients. Early revision of the distal anastomosis, and shortening the conduit, was performed early postoperatively in 2 patients. So far, 24 out 26 survivors of the first stage underwent a bi-directional cavopulmonary anastomosis after a mean interval of 4.3 plus or minus 1.4 months. Of these, 3 required a semi-urgent second stage of palliation because of worsening cyanosis, with one patient dying after the second stage. Completion of the Fontan circulation by insertion of an extracardiac conduit was performed in 8 patients at the mean age of 19.8 plus or minus 2.2 months. We were able to achieve biventricular repair in 1 patient, with aortic atresia, hypoplastic arch and ventricular septal defect, 4.3 months after the initial palliative procedure. Overall survival of the whole cohort of 32 patients was 78.9%, plus or minus 7.8%, at 5 months, and 74.3%, plus or minus 8.6%, up to 25 months.ConclusionsThe introduction of the conduit placed from the right ventricle to the pulmonary arteries has led to an improved outcome in the complex entity of hypoplastic left heart syndrome and its variants. Stenosis of the conduit, nonetheless, may account for significant interstage morbidity, and often requires intervention or early installation of the second stage of palliation.

Use of a saphenous venous homograft for constructing a conduit from the right ventricle to the pulmonary arteries in the Norwood procedure

2004 ◽  
Vol 14 (3) ◽  
pp. 325-327 ◽  
Author(s):  
Luca A. Vricella ◽  
Jane E. Crosson ◽  
Duke E. Cameron
Keyword(s):  
Birth Weight ◽  
Right Ventricle ◽  
Pulmonary Arteries ◽  
Operative Management ◽  
Norwood Procedure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Pulmonary Arterial ◽  
The Right ◽  
Left Heart Syndrome

The use of a conduit of polytetrafluoroethylene placed between the right ventricle and the pulmonary arteries as source of pulmonary arterial supply during the first stage of palliation for the hypoplastic left heart syndrome has facilitated post-operative management and resulted in decreased mortality. We describe here the use of a cryopreserved saphenous vein inserted in reversed direction to create the connection between the right ventricle and the pulmonary arteries in a neonate with low birth weight undergoing the modified Norwood procedure.

Pathological changes and myocardial remodelling related to the mode of shunting following surgical palliation for hypoplastic left heart syndrome

2008 ◽  
Vol 18 (4) ◽  
pp. 415-422 ◽  
Author(s):  
Massimo A. Padalino ◽  
Chiara Castellani ◽  
Silvia Toffoli ◽  
Mila Della Barbera ◽  
Ornella Milanesi ◽  
...  
Keyword(s):  
Extracellular Matrix ◽  
Right Ventricle ◽  
Hypoplastic Left Heart Syndrome ◽  
Pulmonary Arteries ◽  
Left Heart ◽  
Pulmonary Shunt ◽  
Hypoplastic Left Heart ◽  
Surgical Palliation ◽  
The Right ◽  
Left Heart Syndrome

AbstractBackgroundThe modification of placing the shunt from the right ventricle to the pulmonary arteries, also known as Sano procedure, has allegedly improved results over the short term in surgical palliation of hypoplastic left heart syndrome with the Norwood procedure. With this in mind, we reviewed autopsied specimens from neonates and children who did not survive after either a classic arterio-pulmonary shunt, or the modified procedure with the shunt placed from the right ventricle to the pulmonary arteries, so as to evaluate the pathological substrates of the remodelling of the systemic right ventricle, assessing any differences induced by the 2 techniques.MethodsWe obtained the hearts from 11 patients with neonatal diagnosis of hypoplastic left heart syndrome who died after the first or second stages of the Norwood sequence of operations, comparing them with 6 normal hearts matched for age and weight. Macroscopic, microscopic and morphometric analysis were performed on each specimen, evaluating the diameter of the myocytes, extracellular matrix remodelling in terms of fibrosis and type of collagen, and vascularization in terms of capillary density.ResultsHypertrophy of the myocytes was significantly increased in the hearts from patients having either a classic arterio-pulmonary or the ventriculo-pulmonary modification of the shunt compared to controls (p < 0.05). Myocardial fibrosis was increased in those having a shunt placed from the right ventricle to the pulmonary arteries when compared to the other 2 groups. The ratio of collagen I to collagen III was similar in those undergoing a classic arterio-pulmonary shunt compared to controls (0.94), but was lower in those having a shunt placed from the right ventricle to the pulmonary arteries (0.61), with an increase in collagen type III. The density of capillaries was lower in those who had undergone a classic arterial shunt when compared to the others.ConclusionWe have shown greater remodelling of the ventricular myocardial extracellular matrix in patients having a shunt from the right ventricle to the pulmonary arteries when compared to those having a classic arterio-pulmonary shunt, with this remodelling progressing even after the neonatal period. This may influence a later suboptimal ventricular performance.

Implantation of stents as an alternative to reoperation in neonates and infants with acute complications after surgical creation of a systemic-to-pulmonary arterial shunt

2008 ◽  
Vol 18 (2) ◽  
pp. 177-184 ◽  
Author(s):  
Michael Kaestner ◽  
Ronald P. Handke ◽  
Joachim Photiadis ◽  
Matthias Sigler ◽  
Martin B.E. Schneider
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Interventional Procedure ◽  
Pulmonary Arteries ◽  
Emergency Situation ◽  
Interventional Treatment ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Acute Complications ◽  
The Right ◽  
Left Heart Syndrome

AbstractCreation of a systemic-to-pulmonary shunt is still the firstline treatment in neonates with duct-dependent pulmonary circulation, or in patients with hypoplastic left heart syndrome as a part of the first stage of the Norwood sequence. Acute complications after such surgery, such as stenosis, thrombosis, or kinking, are potentially lifethreatening. These complications require immediate revision or exchange of the shunt. In this report, we discuss interventional treatment as an alternative to surgery in 5 patients with acute stenosis or complete occlusion of a shunt.The age of the patients ranged from 12 to 62 days, with a median of 30 days, and their weights ranged between 2.2 and 4.6 kilogrammes, with a median of 3.2 kilogrammes. In 3 patients, the shunts were central or of modified Blalock-Taussig type, while the 2 patients with hypoplastic left heart syndrome had shunts from the right ventricle to the pulmonary arteries. We implanted 6 coronary arterial and 2 peripheral stent systems. The diameter of the balloon used for implantation had a ratio to the shunt of 0.9. to 1.All shunts were successfully reopened by stenting. During follow-up, 3 patients underwent further procedures on an elective basis. We have one patient on the waiting list for further surgical intervention, but one patient died of septicaemia unrelated to the interventional procedure one month after implantation.In our limited experience, implantation of stents is an effective and long-lasting treatment for complications of shunts in an emergency situation.

The management of hypoplastic left heart syndrome with a right aortic arch

2006 ◽  
Vol 16 (5) ◽  
pp. 504-506 ◽  
Author(s):  
Gregory H. Tatum ◽  
Victor O. Morell ◽  
Sang C. Park
Keyword(s):  
Right Ventricle ◽  
Aortic Arch ◽  
Hypoplastic Left Heart Syndrome ◽  
Pulmonary Arteries ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Cavopulmonary Anastomosis ◽  
Reliable Source ◽  
The Right ◽  
Left Heart Syndrome

The combination of hypoplastic left heart syndrome and a right-sided aortic arch is extremely rare and lethal. To the best of our knowledge, no patient with this combination has previously been reported as surviving initial palliation. The anatomic variant is associated with abnormalities in the arteries branching from the aortic arch, making it difficult to construct a reliable source of flow of blood to the lungs. We present here a patient with this combination who survived an initial Damus-Kay-Stansel procedure combined with placement of a conduit from the right ventricle to the pulmonary arteries, and who has subsequently undergone a successful bidirectional cavopulmonary anastomosis. We believe that the conduit placed from the right ventricle provides the most reliable source of flow of blood to the lungs at the time of initial palliation in this usual combination of cardiac lesions.

A 10-year single-centre experience in percutaneous interventions for multi-stage treatment of hypoplastic left heart syndrome

2013 ◽  
Vol 24 (1) ◽  
pp. 54-63 ◽  
Author(s):  
Tomasz Moszura ◽  
Pawel Dryzek ◽  
Sebastian Goreczny ◽  
Anna Mazurek-Kula ◽  
Jacek J. Moll ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Pulmonary Artery ◽  
Hypoplastic Left Heart Syndrome ◽  
Stent Implantation ◽  
Fontan Operation ◽  
Pulmonary Arteries ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Percutaneous Interventions ◽  
Left Heart Syndrome

AbstractObjectivesThe purpose of this paper is to report our 10 years of experience of interventional treatment of patients with hypoplastic left heart syndrome and to focus on the frequency, type, and results of percutaneous interventions during all the stages of palliation, considering the different techniques, devices, and complications.BackgroundConstant progress in surgical treatment of congenital heart defects in the last decade has significantly improved the prognosis for children with hypoplastic left heart syndrome. However, morbidity and mortality remain relatively high. Modern interventional procedures complement or occasionally replace surgical treatment.MethodsBetween January, 2001 and December, 2010, 161 percutaneous interventions were performed in 88 patients with hypoplastic left heart syndrome. Patients were divided into four groups: (a) before the first surgical treatment including hybrid approach, (b) after first-stage Norwood operation, (c) after second-stage bidirectional Glenn operation, and (d) after third-stage Fontan operation.ResultsPercutaneous interventions resulted in statistically significant changes in pulmonary artery pressures, vessel diameters, and O2saturation. Complications occurred in 4.3% of interventions and were related mainly to stent implantation in stenosed pulmonary arteries.ConclusionsPercutaneous interventions may result in haemodynamic stability and reduction in the number of operations. They may result in significant changes in pulmonary artery pressures, vessel diameters, O2saturation, with a low rate of complications, which are mainly related to stent implantation in the pulmonary arteries.

scholarly journals Decreasing Interstage Mortality After the Norwood Procedure: A 30‐Year Experience

2020 ◽  
Vol 9 (19) ◽  
Author(s):  
Michelle Kaplinski ◽  
Richard F. Ittenbach ◽  
Mallory L. Hunt ◽  
Donna Stephan ◽  
Shobha S. Natarajan ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricle ◽  
Hypoplastic Left Heart Syndrome ◽  
Monitoring Program ◽  
Norwood Procedure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Interstage Mortality ◽  
The Right ◽  
Left Heart Syndrome

Background The superior cavo‐pulmonary connection was introduced at our institution in 1988 for infants undergoing surgery for hypoplastic left heart syndrome. Patients with hypoplastic left heart syndrome remain at high risk for mortality in the time period between the Norwood procedure and the superior cavo‐pulmonary connection. The primary objectives of this study were to compare interstage mortality across 4 eras and analyze factors that may impact interstage mortality. Methods and Results Patients with hypoplastic left heart syndrome who underwent the Norwood procedure, were discharged from the hospital, and were eligible for superior cavo‐pulmonary connection between January 1, 1988, and December 31, 2017, were included. The study period was divided into 4 eras based on changes in operative or medical management. Mortality rates were estimated with 95% CIs. Adjusted and unadjusted logistic regression models were used to identify risk factors for mortality. There were 1111 patients who met the inclusion criteria. Overall, interstage mortality was 120/1111 (10.8%). Interstage mortality was significantly lower in era 4 relative to era 1 (4.6% versus 13.4%; P =0.02) during the time that age at the superior cavo‐pulmonary connection was the lowest (135 days; P <0.01) and the interstage monitoring program was introduced. In addition, use of the right ventricle to pulmonary artery shunt was associated with decreased interstage mortality ( P =0.02) and was more routinely practiced in era 4. Conclusions During this 30‐year experience, the risk of interstage mortality decreased significantly in the most recent era. Factors that coincide with this finding include younger age at superior cavo‐pulmonary connection, introduction of an interstage monitoring program, and increased use of the right ventricle to pulmonary artery shunt.

Sign in / Sign up

Export Citation Format

Share Document