Cyanotic Congenital Heart Disease

[Intro: 110 BPM Swing. The track opens with a creepy, wheezing accordion chord.] [The Preamble: The tuba begins its bouncing bassline] extremes of some of the acyanotic like critical PS, aortic coarctation, may cause cyanosis. [Act 1 / Tetralogy: The piano bursts into a fast, chaotic ragtime rhythm. The Ringmaster barks the "Fallot" repetitions with absolute manic energy, pacing the stage.] TETRALOGY OF FALLOT Fallot Fallot Fallot the most common cyanotic congenital heart disease the anterior deviation of the infundibular septum so; pulmonary stenosis, RVH, aortic override, and a VSD Many also have ASD or PFO, initially may be acyanotic single S2, RVH association with right-sided aortic arch, 22q11.2 deletion, Holt-Oram, trisomies 21, 18, and 13 Minimally, neonates with normal saturations-"pink tet behave more like large VSD with heart failure and poor growth by 4 to 6 weeks Severe obstruction Cyanosis blue tet "tet spells," sudden onset or worsening of cyanosis during crying or dehydration Loud systolic ejection murmur from pulmonary stenosis, not VSD If unrepaired, progressive stenosis and worsening RVH and cyanosis Prenatal Diagnosis with fetal echo "boot-shaped" heart due to RVH Comfort the patient, remove stressors Flex knees or place in squatting position Give fluid and Morphine after a hypercyanotic episode, Surgical repair [Act 2: The rhythm shifts to a dizzying, fast-paced waltz. The accordion wails in the background. The vocalist sings this section like a twisted sea shanty.] TRANSPOSITION OF THE GREAT ARTERIES TRANSPOSITION TRANSPOSITION POSITION the most common cyanotic lesion present in the newborn Without mixing of the two circulations, death occurs Mixing with ASD, VSD, PDA Quiet tachypnea, single S2 Increased pulmonary vascularity, RVH the narrow superior mediastinum , an egg on a string PGE1 to ductal patency Complete surgical repair within the first 2 weeks [Act 3 / The Atresia & The Trunk: The drums strip back to just a snapping rim-click and a hi-hat..] TRICUSPID ATRESIA, TRICUSPID TRICUSPID results in a hypoplastic right ventricle A PDA or VSD is necessary for survival Infants usually severely cyanotic and asingle S2 LVH and a superior QRS axis Decreased pulmonary blood flow Surgery needed TRUNCUS ARTERIOSUS TRUNCUS TRUNCUS TRUNCUS A single arterial trunk with a large VSD Varying degrees of cyanosis depend on the amount of pulmonary blood flow signs of heart failure develop bounding Peripheral pulses, single S2 may be a systolic ejection click ventricular hypertrophy, increased pulmonary blood Narrowed mediastinal silhouette anticongestive medications, Surgical repair [Act 4 / The Snowman: The piano plays high, icy, twinkling notes. A slide whistle zips upward on the word "Snowman.] TOTAL ANOMALOUS PULMONARY VENOUS RETURN ANOMALOUS pulmonary veins return to the right side An atrial-level communication is required for survival without obstruction, minimal cyanosis a widely split S2 and a systolic ejection murmur a mid-diastolic murmur of the tricuspid poor Growth with obstruction, there is cyanosis, marked tachypnea and dyspnea, and right-sided heart failure, hepatomegaly. may be no murmur or changes in S2. RVH the heart is normal or mildly enlarged with varying degrees of pulmonary edema "Snowman in a snowstorm" surgically treated [Act 5 / The Hypoplastic: The upbeat circus rhythm suddenly slows down into a heavy, dragging dirge. The tuba plays long, groaning notes.] HYPOPLASTIC LEFT HEART SYNDROME HYPOPLASTIC HYPO the most common cause of death from cardiac defects in the first month failure of normal development of the mitral or aortic valve A small left ventricle and hypoplasia of the aorta dependent on right-to-left shunting As the PDA constricts, the infant becomes critically ill heart failure, weak Pulses, single and loud S2 usually, no murmur Cyanosis may be minimal, low cardiac output gives a grayish color to skin RVH and pulmonary edema PGE1 to PDA Three Staged Surgical repair [Act 6 / The Ebstein Finale: The manic 110 BPM circus swing crashes back in at full volume! Cymbals crash, the accordion screams, and the Ringmaster belts out the final anomaly like the grand finale of a chaotic show.] EBSTEIN ANOMALY EBSTEIN EBSTEIN... downward displacement of the tricuspid valve, tricuspid regurgitation, a larger right atrium and smaller right ventricle bimodal distribution in infancy, in adolescence or adulthood. severely displaced, severe tricuspid valve regurgitation causes cyanosis in the neonate can be associated with pulmonary atresia extreme cardiomegaly due to right atrial dilation may present in utero with fetal hydrops due to severe regurgitation Respiratory failure due to lung hypoplasia holosystolic tricuspid regurgitation murmur Associated with Wolff-Parkinson-White [End: Total Sience]