Presentation on theme: "Cyanotic Heart Lesions"— Presentation transcript:
1 Cyanotic Heart Lesions Case presentation (pink kid turns blue, blue ki)d comes outWhat would you think aboutWhere does cyanotic heart disease come in?ing Dr. MeadowsHow would you approach this patient?Indications/ContraindicationsWhich kids to start PGENeonatal Intensive Care NurseryNight Curriculum Series
2 Cyanosis Arterial saturation less than 90% and a PO2 less than 60 torr In all cyanotic heart lesions the amount of cyanosis seen is dependent on the amount of pulm blood flowDecreased PBF- increased cyanosisIncreased PBF- minimal cyanosis but CHF may developWith 100% oxygenPO2 >250 is not congenital heart diseasePO2 <100 is cardiac diseaseTurn into questions
3 5 “T’s” Most common cyanotic lesions of the newborn Tetralogy of FallotTransposition of the Great ArteriesTruncus ArteriosusTotal Anomalous Venous ReturnTricuspid AtresiaMake this a question and animate it
4 General Sources of Arterial Saturation Decreased Pulmonary Blood FlowAdmixture LesionsIncreased Pulmonary Blood FlowTetralogy of FallotTransposition of Great VesselsTruncious ArteriosusTricuspid AtresiaAnomalous pulmonary venous returnPulmonary Atresia
5 PGE For cyanotic heart lesions with reduced blood flow RE-opens PDA and prevents it from closingAllows partially desaturated systemic arterial blood to enter the pulmonary artery and be oxygenatedInitial dose 0.1mg/kg/minSide effects- apnea, fever, hypotensionAsk what is PGE? How does it work? And What are potential Complications of it?
6 Complete Transposition of the Great Arteries 5% of all CHDBoys 3:1Most common cyanotic condition that requires hospitalization in the first two weeks of life
7 Complete Transposition of the Great Arteries Aorta arises from the right ventriclePulmonary artery arises from the left ventricle
8 Complete Transposition of the Great Arteries Complete separation of the 2 circuitsHypoxemic blood circulating in the bodyHyperoxemic blood circulating in the pulmonary circuit
9 Complete Transposition of the Great Arteries Defect to permit mixing of 2 circulations- ASD, VSD, PDA.VSD is present in 40% of casesNecessary for survival
10 Clinical Symptoms Depend on anatomy present No mixing lesion and restrictive PFOProfound hypoxiaRapid deteriorationDeath in first hours of lifeAbsent respiratory symptoms or limited to tachypneaSingle second heart sound, no murmursanimate
11 Clinical Symptoms Mixing lesion present (VSD or large PDA) Large vigorous infantCyanoticLittle to no resp distressMost likely to develop CHF in first 3-4 months of lifeexcessive sweating (a cold, clammy sweat often noted during feeding); poor feeding, slow weight gain, irritability or lethargy, and/or rapid breathinganimate
12 CXR Egg shaped cardiac silhouette Narrow superior mediastinum Describe the findings on this CXR, and animate
13 Management Prostaglandin to establish patency of the ductus arteriosus Increases shunting from aorta into the pulmonary arteryIncreases pulmonary venous return distending the left atriumFacilitates shunting from the left to the right atrium of fully saturated blood across the foramen ovale.Ask what physiologically happens
14 ManagementTherapeutic balloon atrial septostomy (Rashkind Procedure) if surgery is not going to be performed immediatelyImproves mixing and pulmonary venous return at the atrial level
15 TreatmentSurgery consists of switching the right and left sided structures at the atrial level, at the ventricular level, or at the great artery level.
16 Tetraology of Fallot VSD RVOT Obstruction RVH Overriding aorta Ask what are the 4 compents and animate
17 Two Important Abnormalities NonRestrictive VSDLarge enough to equalize pressures in both ventriclesDegree of RVOTBecause of the non-restrictive VSD, systolic pressures in the RV and the LV are identical.Depending on the degree of RVOT obstruction, and L-R, bidirectional, or r-L shunt is present.Example:Mild RVOT- there is a left to right shunt and infant is acyanoticSevere RVOT- there is a predominant R-L shunt and the infant is cyanoticDegree of cyanosis depends on the degree of right outflow tract obstruction
18 Clinical Presentation of Cyanotic TOF Cyanosis, clubbing, dyspnea on exertion, squatting, hypoxic spells.Loud systolic ejection murmur, systolic thrill at middle LSBSoft murmurs are associated with less blood flow and more hypoxia
19 CXR- boot shapedAsk what are the classic CXR findings
20 Hypoxic Spell (“TET Spell”) Peak incidence of 2-4 monthsCharacterized by:Hyperapnea (Rapid and deep respirations)Irritability and prolonged cryingInc cyanosisDecreased heart murmur
21 PathophysiologyLower SVR or inc resistance of RVOT can increase the R-L shuntStimulates the respiratory center to produce hyperapneaResults in an increase in systemic venous returnIn turn, increases R-L shunt through VSD
22 TET Spell Treatment Hold infant in knee-chest position Morphine Sodium bicarbonate to treat acidosis- decreases resp stimulating effect of acidosisVasoconstrictor (phenylephrine)Propranolol
23 Treatment Early surgical repair depending on pt’s weight VSD is closed and obstructing ventricular muscle is removed
24 Total Anomalous Pulmonary Venous Return The pulmonary veins drain into the RA or its venous tributaries rather than the LAA interatrial communication (ASD or PFO) is necessary for survivalPulmonary venous return reaches the RASystemic and pulmonary venous blood are completely mixed
25 4 Types SupracardiacCommon pulmonary vein drains into the SVC via the left SVC and left innominate vein.
26 2. CardiacThe common PV drains into the coronary sinus
27 3. InfracardiacThe common PV drains into the portal vein, ductous venosus, hepatic vein, or IVC.Infracardiac-type TAPVC. Pulmonary venous blood draining through the liver to reach the IVC and right atrium.
29 Clinical Signs for Unobstructed Veins Mild cyanosis, signs of CHF in infancy, history of pneumoniaWidely split S2, Grade 2-3/6 systolic murmur heard at the ULSBCXR- marked cardiomegaly
30 Clinical Signs for Obstructed Veins Profound desaturationAcidosisPGE1 administration does not improve oxygenation because elevated pulmonary pressures in the right side of the heart (due to obstructed pulmonary outflow) will result in right to left shunting across an open ductus further decreasing arterial saturation.
31 Treatment Digitalis and diuretics to treat heart failure Intubation and inc PEEP for those with severe pulm over loadCorrective surgery
32 Tricuspid Atresia Tricuspid valve is absent RV and PA are hypoplastic Associated defects- ASD, VSD, or PDA (necessary for survival)Dilation of LA and LVEssentially singleventricle physiologyLA and LV are large since they handle both systemic and pulmonary venous return
33 Clinical Signs Severe cyanosis, poor feeding, tachypnea Single S2, grade 3/6 systolic murmur at LLSB if VSD is presentCXR- boot shaped heart
34 Treatment PGE IV infusion Blalock-Taussig shunt in infancy systemic to pulmonary arterial shuntProvide stable blood flow to the lungsA gortex tube is sewen between the subclavian artery and the right pulmonary arteryPGE infusion in severely cyanotic lesions to maintain patency of PDA
35 Bidirectional GlennSuperior vena cava is connected to the pulmonary arteriesIVC continues to be connected to the heart
36 Fontan Procedure Redirects IVC to lungs Now oxygen-poor blood from upper and lower body flows through the lungs without being pumped and corrects cyanosisThe single ventricle is only responsible for supplying blood to the body.
37 Truncus Arteriosus A single trunk leaves the heart Gives rise to pulm, systemic, and coronary circulationsLarge VSD is always present
38 Clinical Signs Cyanosis immediately after birth Early signs of CHF 2-4/6 systolic murmur at LSB suggestive of VSD
39 Treatment Anticongestive medications (diuretics and digitalis) Corrective surgeryVSD is closedPulmonary artery is separated from the truncusContinuity is then established between the right ventricle and the pulmonary artery utilizing a valved homograft conduit