0 valutazioniIl 0% ha trovato utile questo documento (0 voti)
102 visualizzazioni6 pagine
Congenital heart disease requires evaluation with ECG, CXR, and hyperoxia testing. Cyanotic heart diseases involve ductal-independent mixing lesions like truncus arteriosus and transposition of the great arteries, which present with cyanosis and require surgical repair. Ductal-dependent lesions involve tricuspid atresia, tetralogy of Fallot, and Ebstein's anomaly, which require keeping the ductus arteriosus open until surgery. Hypoplastic left heart syndrome has ductal-dependent systemic blood flow and presents with congestive heart failure, requiring palliative surgery.
Congenital heart disease requires evaluation with ECG, CXR, and hyperoxia testing. Cyanotic heart diseases involve ductal-independent mixing lesions like truncus arteriosus and transposition of the great arteries, which present with cyanosis and require surgical repair. Ductal-dependent lesions involve tricuspid atresia, tetralogy of Fallot, and Ebstein's anomaly, which require keeping the ductus arteriosus open until surgery. Hypoplastic left heart syndrome has ductal-dependent systemic blood flow and presents with congestive heart failure, requiring palliative surgery.
Congenital heart disease requires evaluation with ECG, CXR, and hyperoxia testing. Cyanotic heart diseases involve ductal-independent mixing lesions like truncus arteriosus and transposition of the great arteries, which present with cyanosis and require surgical repair. Ductal-dependent lesions involve tricuspid atresia, tetralogy of Fallot, and Ebstein's anomaly, which require keeping the ductus arteriosus open until surgery. Hypoplastic left heart syndrome has ductal-dependent systemic blood flow and presents with congestive heart failure, requiring palliative surgery.
o ECG, CXR, and hyperoxia test (measuring preductal (radial a.) arterial blood gas on room air and then repeating on FIO2 100%. Interpretation helps narrow diagnosis Cyanotic Heart Disease (Ductal Independent Mixing Lesions) TTTT o Truncus Arteriosus Background: Single arterial vessel, therefore complete mixing of systemic and pulmonic blood usually has ventricular septal defect, 30% have right sided aortic arch Associated with 22q11 microdeletion DiGeorges syndrome may hypocalcemia Clinical Manifestations Moderate Cyanosis, CHF develops in weeks, biventricular hypertrophy on ECG Holosystolic Murmur at L sternal border Single Loud S2 b/c theres only 1 valve Treatment Anticongestion = digoxin & diuretics Surgical repair close VSD, and make conduit b/n both aorta and pulm artery o d-Transposition of Great Arteries Background: Aorta arises from RV, Pulmonary A. arises from LV operates in Parallel #1 cyanotic HD presenting in neonates, 5% of all congenital heart disease Most have intact ventricular septum ASD, VSD, or PDA that allows mixing is required for survival Clinical Manifestions Cyanosis present from birth, Single Loud S2, Right ventricular heave, If ventricular septum defect, then harsh holosystolic murmur lower left sternal border Right ventricular hypertrophy seen, right axis deviation on ECG, Egg-shaped heart on X-ray Treatment PGE1 to keep PDA open Rashkind balloon atrial septostomy atrial mixing of blood Surgical repair switch back o Total Anomalous Pulmonary Venous Connection without Obstruction (TAPVC) Background: Rare lesion, where all pulmonary venous return is directed to the Right Atrium o May drain to: Supracardia: SVC (50%), Cardiac: Directly to RA (20%), Infradiaphragmatic: Portal or Hepatic (20%), Mixed (10%). Without obstruction refers to the site where the anomalous vein enters a vessel ie there is no constriction at this site. Therefore there is increased Pulmonary blood flow, mild Pulmonary HTN, and Moderate Cyanosis Clinical Manifestations Findings similar to Atrial septal defect Right ventricular heave, wide and fixed split S2 Systolic ejection murmur at Left upper sternal border Cardiomegaly, increased pulmonary vasculature on CXR, ECG shows right axis deviation and Right Ventricular Hypertrophy Treatment Rx of CHF Surgically redirect vessels into Left Atrium Necessary in 1st month of Life Cyanotic Congenital Heart Disease: Lesions with Ductal-Dependent Pulmonary Blood flow o Tricuspid Atresia Background: No connection b/n RA and RV, leads to hypoplasia or absence of Right ventricle 30% have transposition of great arteries, 90% have VSD (this allows blood to flow from the left ventricle to both the Pulm A. & Aorta Most also have pulmonic stenosis The only way for systemic blood to get to the Left side (and therefore the Pulm A. and Aorta is through a Patent Foramen Ovale or ASD Clinical Manifestations Cyanosis is severe, poor feeding, tachypnea over first 2 weeks of life VSD loud holosystolic murmur at left lower sternal border if present PDA continuous murmur if present ECG superior axis and Left Ventricular Hypertrophy Treatment PGE1 to keep PDA open to maintain Pulmonary flow balloon atrial septostomy, if atrial defect not sufficient Blalock-Taussig Shunt Gor-Tex conduit b/n subclavian Artery and Pulmonary A. o Pulmonic Atresia with Intact Ventricular Septum Background: Rare defect with pulmonary valvular and infundibular atresia with right ventricular and tricuspid valve hypoplasia o ie. Basically the Right Ventricle is unable to produce any pulmonary outflow so RV is hypertensive and have Tricuspid Regurgitation. Dependent on PDA for survival In some cases Coronary perfusion is dependent on Right Ventricle! Therefore decreasing RV hypertension can lead to MI and death Clinical Manifestations Extremely cyanotic, tachypneic, Tricuspid Regurgitation murmur on left sternal border Continuous PDA murmur ECG LV hypertrophy, leftward axis Treatment PGE1 to keep PDA open to maintain Pulmonary flow Must check coronary arteries before surgery via catheterization If coronaries not RV dependent, then RV to pulmonary artery connection is made o Tetralogy of Fallot Background: #1 cause of Cyanosis presenting during 3rd week of life 4 defects: VSD, Right ventricular outflow obstruction, Right ventricular hypertrophy, and overriding large aorta Clinical Manifestations Cyanotic due to right to left shunting across VSD and decreased Pulmonary flow Tet Spells periodic cyanosis and agitation caused be increase in RV outflow tract resistance, increasing the R L shunt. Last minutes to hours, may resolve or progress to death exam: RV heave felt, Systolic ejection murmur in L upper sternum due to RV outflow obstruction PDA cont murmur, VSD holosystolic murmur at Left lower sternal border ECG shows RA dilation, RV hypertrophy 25% have right sided arch Treatment Tet-spells Rx by decreasing R L shunting, by increasing systemic vascular resistance, and dec pulm vascular resistance o Supplimental O2, vagal maneuvers, morphine sulfate, vasoconstrictors, beta- blockers and volume administration, also bring the childs knees to their chest increasing systemic resistance and decreasing the shunt from Right to Left. o pull knees of child to chest decreases preload, increases systemic resistence o Ebsteins Anomaly Background Extremely rare, where leaflets are displaced into RV cavity Results in hypoplasia of RV, tricuspid regurg and/or stenosis 80% have Patent Foramen Ovale, with R L shunt Massively dilated RA Associated with Wolff-Parkinson-White (WPW) syndrome Clinical Manifestations Cyanosis and CHF in first few days of life Widely fixed S2 and Tricuspid Regurg at L lower sternal border ECG shows Right Bundle Branch Block Delta Waves due to WPW syndrome CXR MASSIVE cardiomegaly, with enlarged RA Treatment PGE1 to keep PDA open to maintain Pulmonary flow CHF therapy with Digoxin and diuretics Surgical repair of Tricuspid has had poor results Cyanotic Congenital Heart Disease: Lesions with Ductal Dependent Systemic Blood Flow o Hypoplastic Left Heart Syndrome (HLHS) Background #1 cause of death from congenital HD during 1st month Syndrome with: Hypoplasia of LV, Aortic Valve stenosis or atresia, Mitral Valve stenosis or atresia and hypoplasia of ascending aortic with coarctation of aorta. All combine to reduce flow through the Left side Causes L R shunt at Atrial level & R L shunt at Ductus Arteriosus Clinical Manifestations As ductus close CHF, moderate cyanosis, tachypnea, pulmonary rales and hepatomegaly Poor or absent peripheral pulses S3 and Loud single S2 Treatment PGE1 to keep PDA open to maintain flow to the Aorta from right side Norwood palliation: restores unobstructed systemic blood flow o Interrupted Aortic Arch Background Aortic arch is not complete and ends in: Type A: beyond L subclavian, Type B: b/n L subclavian and L common carotids, Type C: b/n L common carotids and brachiocephalic arteries All flow distal to the dead-end must come from PDA (R L) Also associated with DiGeorges syndrome due to 22q11 microdeletion Clinical Manifestations Pulmonary edema Presentation similar to Critical Coarctation of Aorta (below) Treatment PGE1 to keep PDA open to maintain systemic flow Surgery to reanastomose aortic segments o Total Anomalous Pulmonary Venous Connection with Obstruction Background see above Presence of Severe Pulmonary Edema differentiates from w/o obstruction Clinical Manifestations TAPVC w/ obstruction presents with extreme cyanosis, tachypnea, and dyspnea RV heave, narrowly split S2, Ventricular gallop (S3) ECG R axis deviation, RA dilation, RVH CXR pulmonary edema Treatment PGE1 should not be given as pulmonary flow is already overflowed Management of CHF and Pulmonary edmema Surgical redirection of aberrant vessels into left atrium Acyanotic Congenital Heart Disease o Atrial Septal Defects Background 3 types: o Ostium secundum: midportion of atrial septum o Ostium primum: defect in low atrial septum o Sinus venosus: defect at junction of RA and SVC or IVC Usually results in L R shunting, increasing Pulm blood flow Clinical Manifestations Usually asymptomatic RV heave often present Systolic ejection murmur in Pulmonic area S2 wide and constantly split Treatment Ostium Secundum usually spontaneously close Ostium Primum and sinus venosus require surgical closure o Ventricular Septal Defects Background #1 congenital defect, 26% of all 5 types: conoventicular, muscular, inlet, conoseptal hypoplasia, malalignment Clinical Manifestations Small shunt has no symptoms Larger, gives growth failure, CHF, chronic lower respiratory infection With Eisenmenger physiology (R L) presents with SOB, dyspnea, chest pain, and cyanosis. Smaller defect = louder murmur If pulmonary vascular obstruction RV heave, ejection click, short systolic ejection murmur, diastoloic murmur of pulmonary valve insufficiency, Loud S2 Treatment Most close without intervention Rx CHF May require surgery if large o Common Atrioventricular Canal Background Endocardial cushion defect Deficiency of both atrial and ventricular septa and of Mitral and tricuspid valves Severity varies widely depeding on extend of defect. Approx 1/3 of Downs syndrome have this; L R shunt at atrial and ventricular level Clinical Manifestations Pulmonary HTN may develop Presents with tachypnea, dyspnea, and poor feeding Blowing holosystolic murmur at L lower sternal border due to VSD and Fixed wide S2 due to ASD ECG right axis deviation, RA dilation, LA dilatation Treatment Surgical repair o Patent Ductus Arteriosus Background 10% of Congenital heart disease, 2:1 female, higher in premies, connects Pulmonary a to Aorta just distal to Left Subclavian a. Clinical Manifestations Small no symptoms Larger L R: CHF, slow growth, repeated Lower Resp tract infections Reversal to R L: result of high pulm vasc resistance, causes SOB, dyspnea and Cyanosis Continuous machinery murmur Treatment Indomethacin closes PDA by decreasing PGE1 levels Usually closes by 1 month, if not surgery is required o Coarctation of the Aorta Background Constriction of aorta, usu decending at insertion site of ductus. If in female consider Turners syndrome Aortic valve is Bicuspid in 80%, Clinical Manifestations Weak femoral pulses relative to upper If severe may rely on PDA Systolic ejection murmur at Apex Visualized with Echo Treatment Balloon angioplasty or surgical end-to-end anastomosis o Aortic Stenosis Background Rigid thinkened valvular tissue LV hypertrophy Clinical Manifestations Presents with cardiac collapse or harsh systolic ejection murmur at right upper sternal border and is preceded by ejection click If severe may depend on PDA On CXR see poststenotic dilatation of aorta May see inverted T waves Treatment Open surgical valvotomy or by balloon valvuloplasty o Pulmonic Stenosis Background Pulmonary commissures are fused Clinical Manifestations May cause dyspnea on exertion and angina, Ejection click varies with inspiration Harsh ejection murmur at L upper sternal border Treatment Balloon valvuloplasty
Dark Psychology & Manipulation: Discover How To Analyze People and Master Human Behaviour Using Emotional Influence Techniques, Body Language Secrets, Covert NLP, Speed Reading, and Hypnosis.
Raising Mentally Strong Kids: How to Combine the Power of Neuroscience with Love and Logic to Grow Confident, Kind, Responsible, and Resilient Children and Young Adults