Norwood Procedure in a Patient With Hypoplastic Left Heart Syndrome, Right Aortic Arch, and Right Descending Aorta

Virgilijus Tarutis1, Virgilijus Lebetkevičius1, Kęstutis versockas2, Asta Bliūdžiūtė2, Žydrė Jurgelienė2, Solveiga Umbrasaitė1, Rita Sudikienė3, Daina Liekienė1, Kęstutis Lankutis3, Vidmantas Jonas Žilinskas1, Vytautas Sirvydis11 Vilniaus universiteto Širdies chirurgijos centras, Santariškių g. 2, LT-08661 Vilnius2 Vilniaus universiteto ligoninės Santariškių klinikų Anesteziologijos,intensyviosios terapijos ir skausmo gydymo centras3 Vilniaus universiteto ligoninės Santariškių klinikų Širdies chirurgijos centras,Santariškių g. 2, LT-08661 VilniusEl paštas: [email protected] Įvadas / tikslas Kairiosiosios širdies hipoplazijos sindromas (KŠHS) yra viena sunkiiausių įgimtų širdies ydų. KŠHS turi 1 iš 4000–6000 naujagimių. Negydant mirštamumas pirmaisiais gyvenimo metais būna didesnis kaip 90%. Apžvelgiame pradinę KŠHS chirurginio gydymo patirtį. Ligoniai ir metodai Nuo 2005 m. iki dabar operuoti 9 naujagimiai, turintys KŠHS. 6 pacientai operuoti stabilizavus jų būklę, 3 operuoti nestabilios būklės esant kraujotakos nepakankamumui. 4 pacientams atlikta klasikinė Norwoodo operacija su modifikuota Blalocko ir Taussig jungtimi, likusiems 5 atlikta Norwoodo operacijos Sano modifikacija. Rezultatai Pirmas Norwoodo korekcijų etapas buvo sėkmingas 5 (55,6%) naujagimiams: 3 buvo padaryta modifikuota B-T jungtis, 2 – Sano jungtis. Išvada KŠHS chirurginis gydymas mūsų centre kol kas yra vadinamosios mokymosi kreivės laikotarpio. Norwoodo I etapo korekcijos rezultatai turėtų gerėti padidėjus operacijų skaičiui ir griežčiau atrenkant pacientus, atsižvelgiant į rizikos veiksnius. Pagrindiniai žodžiai: kairiosios širdies hipoplazijos sindromas, Norwoodo operacija Initial experience in first-stage surgical treatment of hypoplastic left heart syndrome Virgilijus Tarutis1, Virgilijus Lebetkevičius1, Kęstutis versockas2, Asta Bliūdžiūtė2, Žydrė Jurgelienė2, Solveiga Umbrasaitė1, Rita Sudikienė3, Daina Liekienė1, Kęstutis Lankutis3, Vidmantas Jonas Žilinskas1, Vytautas Sirvydis11 Vilnius University, Cardiac Surgery Centre, Santariškių str. 2, LT-08661 Vilnius, Lithuania2 Vilnius University Hospital „Santariškių klinikos“,Anesthesiology, Intensive Care and Pain Management Center3 Vilnius University Hospital „Santariškių klinikos“,Cardiac Surgery Centre Santariškių str. 2, LT-08661 Vilnius, LithuaniaE-mail: [email protected] Background / objective Hypoplastic left heart syndrome (HLHS) is one of the most complex congenital heart defects. The incidence of HLHS is 1 in 4000 to 6000 live births. The mortality rate exceeds 90% without treatment in the first year of life. We review the initial experience in the surgical treatment of HLHS at our Centre. Patients and methods There were 9 children operated on for HLHS from the year 2005 till now. Six of them went to operation in hemodinamically stable condition and three had a substantial insufficiency of systemic circulation. Four underwent Norwood I procedure with modified Blalock–Taussig shunt and five had Sano modification. Results The first-stage Norwood procedure was successful in five (55.6%) cases – three with B-T shunt and 2 with Sano. Conclusions Surgical treatment of HLHS remains in the learning curve period at our institution. We expect the rise of survival rate with the increase of case load and a more selective approach considering the risk factors. Key words: hypoplastic left heart syndrome, Norwood procedure

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Early outcomes and computational fluid dynamic analyses of chimney reconstruction in the Norwood procedure

Interactive CardioVascular and Thoracic Surgery ◽

10.1093/icvts/ivz040 ◽

2019 ◽

Vol 29 (2) ◽

pp. 252-259 ◽

Cited By ~ 2

Author(s):

Satoshi Asada ◽

Masaaki Yamagishi ◽

Keiichi Itatani ◽

Yoshinobu Maeda ◽

Satoshi Taniguchi ◽

...

Keyword(s):

Aortic Arch ◽

Hypoplastic Left Heart Syndrome ◽

Fluid Dynamic ◽

Norwood Procedure ◽

Patient Specific ◽

Shear Stresses ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Flow Profiles ◽

Left Heart Syndrome

Abstract OBJECTIVES The ideal configuration of a reconstructed aortic arch in the Norwood procedure for hypoplastic left heart syndrome is still a matter of debate. Chimney reconstruction was developed to avoid postoperative complications and turbulent flow in the aortic arch. This study sought to clarify early outcomes of the procedure and verify its haemodynamic advantages using computational fluid dynamics (CFD). METHODS Fourteen consecutive patients with hypoplastic left heart syndrome or a variant who underwent chimney reconstruction in the Norwood procedure between January 2013 and March 2018 were enrolled. Median age and body weight at the time of operation were 2.5 months and 4.1 kg, respectively. Thirteen patients (93.9%) had been palliated with previous bilateral pulmonary artery (PA) banding. In addition, patient-specific CFD models of neoarches based on postoperative computed tomograms from 6 patients were created and the flow profiles analysed. RESULTS Survival rates at 1, 3 and 5 years were 76.6%, 67.3% and 67.3%, respectively. No patient developed left PA compression by neoaorta, neoaortic dilation or neoaortic insufficiency. Only 2 patients (14.3%) required surgical intervention for recoarctation. Fontan completion was performed on 5 patients. On CFD analysis, all reconstructed aortic arches showed low energy loss (9.16–14.4 mW/m2) and low wall shear stresses. CONCLUSIONS Chimney reconstruction was a feasible technique when homografts were not readily available. CFD analyses underscored the fact that this technique produced excellent flow profiles. Larger studies should be conducted to clarify long-term outcomes.

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Hypoplastic left heart syndrome with right aortic arch, bilateral arterial ducts and origin of the left subclavian artery from the left pulmonary artery

Cardiology in the Young ◽

10.1017/s1047951100005047 ◽

1999 ◽

Vol 9 (3) ◽

pp. 331-334 ◽

Cited By ~ 3

Author(s):

Chandrakant R. Patel ◽

Michael L. Specter ◽

Kenneth G. Zahka

Keyword(s):

Pulmonary Artery ◽

Aortic Arch ◽

Hypoplastic Left Heart Syndrome ◽

Subclavian Artery ◽

Left Subclavian Artery ◽

Left Pulmonary Artery ◽

Right Aortic Arch ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Left Heart Syndrome

AbstractThe rare association, in a left-sided heart with hypoplastic left heart syndrome, of right aortic arch, bilateral patent arterial ducts and origin of the left subclavian artery from the left pulmonary artery are described. Cardiac catheterization was performed because of the abnormal anatomy of the arch noted at echocardiographic examination. This abnormality is of surgical importance when planning the Norwood operation.

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