Presentation on theme: "Cyanotic Heart Disease"— Presentation transcript:
1Cyanotic Heart Disease THE “TERRIBLE T’s”Cyanotic Heart Disease
2GOALSTo define the physiology of the five most common cyanotic defectsTo describe the long term sequelae of the corrective procedure.To describe the PE,ECG, and CXR results for the more common defects.
3INITIAL WORKUP: THE HYPEROXY TEST Give the patient as close to FiO2 of 1 for at least 5 minutes.Obtain pre and postductal ABG’SWhat should your PaO2 be on 100% O2?Is it reasonable to expect to get 100% using a mask or hood?
4CXRMainly we are looking for situs, PBF, cardiac size and noncardiac abnormalities.Boot shaped, apple shaped, box shaped, valentine shaped, egg on it’s side shaped, ball on string shaped, boiled peanut shaped…el toro poo poo.
5ECG Get it! If you can’t read it fax it! Remember most term infants have relative RV dominance so a big LV would be unusual.Unless it’s a significant premie where there hasn’t been time to develop the pulmonary vascular bed. In a premie, RVE is not the norm
6WHAT ARE THEY? Tetralogy of Fallot d-Transposition of the great arteriesTruncus arteriosusTAPVRAtresia’s
7TETRALOGY of FALLOT See handout for basics. The primary concerns after correction are: the degree of Pulmonary insufficiency, the presence of tachydysrhymias, and residual VSD’s, and the presence of significant supravalvular pulmonary stenoses.There seems to be a degree of mild learning disability post op, also ADHD.
8d-TGA The most cyanotic common defect diagnosed in the nursery. The degree of saturation will depend on the degree of mixing of the 2 “parallel” circuits.The mixing sites are: ASD, PDA, and VSD. The more mixing, the higher the “effective pulmonary blood flow”
9EFFECTIVE PULMONARY FLOW Blood going to the lungs which has not been to the lungs in that cardiac cycle.PGE acts to increase mixing at the great vessel level.BAS increases mixing at the atrial levelWe try not to increase mixing at the ventricular level.
10CLINICAL Findings d-TGA CyanosisPossibly CHFIf pulmonary stenosis present, may be very blueS2 normally split in 1/3, narrow and loud in 2/3ECG: RAD with BE. May be wnl in nurseryCXR: egg on string.Narrow medias.^PBF
11D-TGA cont. Cath findings: slides ECHO: classic findings are anterior great vessel gives off CA’s, posterior vessel gives off two branches, may look like circle,circle
12d-TGA TREATMENTPGE: usually all that’s needed to improve mixing and relieve the acid base issues. At least long enough to get the patient to a center.BAS may be necessary if PGE not effective and there’s a delay in correction.MUSTARD: see slideSWITCH
13MUSTARD SEQUELAESinus node dysfunction: 10 yrs out less than 30% in dominant sinus rhythm.Atrial tachyarrhymias.Ventricular arrhymias….sudden deathCoronary perfusion abnormalitiesRV failure
14ASO: follow -upSupravalvular obstruction: most commonly PS with frequent LPA stenosis.Coronary occlusion: occurs early. Had to correct if laterNeo-aortic regurg: more frequently reported. No cases requiring surgical correction but a few have required medical intervention
15TAPVRCyanosis variable and largely dependant on degree of pulmonary venous obstruction.CHF found in 41%, if early probably because of pulm vein obstruction. If later,in those without pn obs.One of the few defects which can give you a big heart and cyanosis. The other is Ebsteins.
16TAPVR cont. ECG: RAD,RVE, reversal of r/s progression,RAE by 3 months Snowman on CXRECHO: usually a smallish LA, abnormalities of venous return(difficult to find any PV’s coming to LA). May find the common vein.
17TAPVRThe patients are not infrequently our sickest as infants. The are profoundly hypoxic and frequently acidotic.The biggest post-op issue is pulmonary vein stenosis. If that’s present, pulmonary HTN is inevitable and extremely difficult to tx.
18TAPVR: ECMOBecause of the extreme condition many of these infant present in, some of them have been felt to have PPHN and have been submitted for ecmo. This was especially true before the availability of color doppler. This meant a number of these patients were in much better condition when they went for repair.
19TRUNCUS ARTERIOSUSA SINGLE GREAT VESSEL ARISES FROM THE HEART AND GIVES OFF THE CA’S,PA’S AND AORTA.19TH MOST COMMON CONGENITAL HEART DEFECTONE OF THE LESIONS ASSOCIATED WITH RIGHT ARCH AND AORTIC OVERRIDE.
20TRUNCUS cont4 basic types based on the source of PBF. The fourth is pulmonary atresia with vsd otherwise known as pseudotruncus.11% of cases have interrupted aortic archThe is associated DiGeorge syndromeThere may be associated pulmonary stenosis
21TruncusIf there is no pulmonary obstruction, the dominant picture is CHF with varying degrees of cyanosisThere may be significant insufficiency of the truncal valves which can have 2-5 cusps.Bounding pulses(it’s like a big PDA), There may be a continous murmur if the PA’s are tight
22TRUNCUS ECG: LVE,RVE OR BE. OCC LAE CXR: RIGHT ARCH 30%, ^PBF ECHO: USUALLY DIAGNOSTIC
23TRUNCUS:Tx Decongestive tx pending surgery Surgery consists of VSD closure and a graft to the PA’s.Early surgery essential.The average age of death untreated is 5 wks.Sequelae:depends on degree of truncal valve insufficiency and pulm artery obstruction