THE “TERRIBLE T’s”

Cyanotic Heart Disease

GOALS

• To define the physiology of the five most common cyanotic defects

• To describe the long term sequelae of the corrective procedure.

• To describe the PE,ECG, and CXR results for the more common defects.

INITIAL WORKUP: THE HYPEROXY TEST

• Give the patient as close to FiO2 of 1 for at least 5 minutes.

• Obtain pre and postductal ABG’S

• What should your PaO2 be on 100% O2?

• Is it reasonable to expect to get 100% using a mask or hood?

CXR

• Mainly 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.

ECG

• 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

WHAT ARE THEY?

• Tetralogy of Fallot

• d-Transposition of the great arteries

• Truncus arteriosus

• TAPVR

• Atresia’s

TETRALOGY 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.

d-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”

EFFECTIVE 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 level

• We try not to increase mixing at the ventricular level.

CLINICAL Findings d-TGA

• Cyanosis

• Possibly CHF

• If pulmonary stenosis present, may be very blue

• S2 normally split in 1/3, narrow and loud in 2/3

• ECG: RAD with BE. May be wnl in nursery

• CXR: egg on string.Narrow medias.^PBF

D-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

d-TGA TREATMENT

• PGE: 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 slide

• SWITCH

MUSTARD SEQUELAE

• Sinus node dysfunction: 10 yrs out less than 30% in dominant sinus rhythm.

• Atrial tachyarrhymias.

• Ventricular arrhymias….sudden death

• Coronary perfusion abnormalities

• RV failure

ASO: follow -up

• Supravalvular obstruction: most commonly PS with frequent LPA stenosis.

• Coronary occlusion: occurs early. Had to correct if later

• Neo-aortic regurg: more frequently reported. No cases requiring surgical correction but a few have required medical intervention

TAPVR

• Cyanosis 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.

TAPVR cont.

• ECG: RAD,RVE, reversal of r/s

• progression,RAE by 3 months

• Snowman on CXR

• ECHO: usually a smallish LA, abnormalities of venous return(difficult to find any PV’s coming to LA). May find the common vein.

TAPVR

• The 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.

TAPVR: ECMO

• Because 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.

TRUNCUS ARTERIOSUS

• A SINGLE GREAT VESSEL ARISES FROM THE HEART AND GIVES OFF THE CA’S,PA’S AND AORTA.

• 19TH MOST COMMON CONGENITAL HEART DEFECT

• ONE OF THE LESIONS ASSOCIATED WITH RIGHT ARCH AND AORTIC OVERRIDE.

TRUNCUS cont

• 4 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 arch

• The is associated DiGeorge syndrome

• There may be associated pulmonary stenosis

Truncus

• If there is no pulmonary obstruction, the dominant picture is CHF with varying degrees of cyanosis

• There 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

TRUNCUS

• ECG: LVE,RVE OR BE. OCC LAE

• CXR: RIGHT ARCH 30%, ^PBF

• ECHO: USUALLY DIAGNOSTIC

TRUNCUS: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