1 / 24

Hypoplastic left heart syndrome and the Rastelli procedure

Hypoplastic left heart syndrome and the Rastelli procedure. Catherine Kim-Gavino, PGY4 Loyola University Medical Center. Admixture Lesions. Mixing of R & L circulations across a large VSD, ASD, PDA Admixed blood flows to pulmonary circulation Cyanotic with increased PBF.

sarno
Download Presentation

Hypoplastic left heart syndrome and the Rastelli procedure

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Hypoplastic left heart syndrome and the Rastelli procedure Catherine Kim-Gavino, PGY4 Loyola University Medical Center

  2. Admixture Lesions • Mixing of R & L circulations across a large VSD, ASD, PDA • Admixed blood flows to pulmonary circulation • Cyanotic with increased PBF

  3. HLHS (Aortic Atresia) • Complex combination of cardiac malformations (8%) • Most common cause cardiac death 1st week of life • 4th most common cardiac malformation within 1st year of life • Behind VSD, d-TGA, TOF • M:F 2:1 • Invariably lethal if left untreated • 25% early cardiac deaths in neonate

  4. Pathophysiology • Hypoplastic/absent LV, small sinuses of Valsalva, small Ao annulus/ascending Ao, LA, • 25% have a small MV or atresia • L-sided outflow tract obstruction • Variable severity • Systemic perfusion of aorta entirely thru PDA@ pulmonary pressures • Occasional ASD or VSD

  5. From www.mayoclinic.org

  6. From www.childrenscentralcal.org

  7. HLHS • During systole: R-L flow through PDA to systemic circulation. • During diastole: L-R flow across PDA and retrograde flow through ascending Ao to perfuse coronaries. • PVR enters LA: restricted flow across atretic MV into hypoplastic LV. • LA blood shunted across interatrial communication (pinhole-sized FO to widely patent ASD). • Restricted FO flow + decreased flow through MV causes PVO obstruction. (typical presentation of neo w/severe CHF). • Nonrestrictive ASD allows larger-volume L-R shunt at atrial level and increased pulm artery BF. From www.childrenscentralcal.org

  8. HLHS • Pulmonary, systemic, coronary circulation depends on RV • Rt-sided HF occurs rapidly, esp as ductus is closing (24-48 hrs) • Dusky infant in failure in first 48 hrs of life = HLHS • Maintain PDA patency with IV PGE1.

  9. Imaging • Prenatal US: diastolic flow reversal in extremely narrow ascending Ao is diagnostic for HLHS. • Real-time echo: • sm, thick walled LV, sm MV w/restricted motion • Dilated RH/PA, lg PDA • Sm ascending Ao • CXR: • Moderate-marked CM • Globular cardiac silhouette suggesting combined chamber enlargement • PV congestion with interstitial/pleural fluid

  10. www.med.yale.edu

  11. From Texas Children’s Hospital www.bcm.edu

  12. Prognosis • CHF and death --> MI secondary to restrictive coronary flow or poor AV mixing. • Universally fatal with 80% patient dying within the first week. • Heart transplantation or staged Norwood procedure are alternative forms of therapy.

  13. Norwood (3 stages) • Stage 1. At birth • Conduit RV to Ao root (Rastelli repair) • Divide PA from RV and ductus • Perfuse PA via Blalock-Taussig shunt • Stage 2. At 6 mos • Construct Glenn shunt (SVC to PA) to reduce volume load on RV • Stage 3. At 18 mos • Extracardiac Fontan. Direct blood from IVC & SVC thru RA to PA

  14. But wait…. • Not all patients undergo this 3-stage palliative procedure. • If LV still functional, both ventricles can participate in cardiac circulation

  15. Our patient, A.R. • 5 yo girl with past h/o resp distress soon after birth • CXR • Further w/u • Large VSD • Subaortic stenosis • Small ascending Ao • Coarctation of aorta

  16. Rastelli surgery • Intracardiac conduit/baffle: • establishes continuity b/w LV and Ao • performed usu in DORV, forms of transposition (particularly w/VSD and subpulmonic stenosis). • Extracardiac conduit: • b/w RV and PAs during repair of TOF w/pulmonary atresia, TA, DORV. • Either a homograft AV w/segment of ascend Ao or a Dacron conduit with porcine AV is used.

  17. Royal Children’s Hospital Melbourne www.rch.org.au

  18. A.R. • Treated with modified Norwood & Rastelli procedure: • Conduit from RV to pulmonary artery (w/AV) • Large baffle across VSD from LV to “neoaorta” • Constructed from Ao arch, descending Ao, MPA. • Ao root is anastomosed to neoaorta. • “Systemic valve” is PV • Closure of ASD • Repair of coarct.

  19. www.heartchina.net

  20. AXIAL FIESTA

  21. LVOT SAG FIESTA RVOT

  22. Complications • Obstruction b/w LV & Ao (VSD level) or other anastomotic sites • Incorrect placement of intracardiac baffle. • If VSD must be enlarged, may get complete HB, necessitating pacemaker. • Calcification of conduit/prosthetic valve ( conduit obstruction). • Obstruction/narrowing within conduit (neointimal fibrotic peel), thrombosis, periconduit abscess . • Pseudoaneurysms at anastomotic sites.

  23. The End This is how I’m feeling, post-call

  24. References • Kersting-Sommerhoffi BA, Seelos KC, Hardy C, Kondo C, Higgins SS, Higgins CB. The Evaluation of Surgical Procedures for Cyanotic Congenital Heart Disease by Using MR Imaging. AJR 1990; 155:259-266, • Bardo DME, Frankel DG, Applegate KE, Murphy DJ, Saneto RP. Hypoplastic Left Heart Syndrome. RadioGraphics 2001; 21:705-717 • Lonergan GL. Congenital Heart Disease. “Radiologic Pathology,” from the Armed Forces Institute of Pathology. Vol 3. 2006-2007. • Kuhn JP, Slovis TL, Haller JO. Caffey’s Pediatric Diagnostic Imaging. 10th ed. Mosby, Philadelphia. 2004

More Related