common pediatric and congenital cardiac abnormalities ...case 1 •1 month old asymptomatic male...
TRANSCRIPT
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Common Pediatric and Congenital Cardiac
Abnormalities – Selected Cases
Brett W Goudie, MD
Pediatric Heart Center
Hasbro Children’s Hospital – Brown University
Providence RI
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Goals
• Review select interesting and overlooked cardiac abnormalities seen in young patients
• Outline the thought process involved in managing children with these abnormalities
• Use echo images and other pictures and sounds to keep everyone awake
• Sadly, again, nothing financial to disclose
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Case 1
• 1 month old asymptomatic male with murmur, no cyanosis, no respiratory distress, no feeding difficulty, normal growth
• PMHx, Fam Hx, Soc Hx, ROS all negative
• Vitals: HR140, RR33, BP 80/46 RUE, 85/48 RLE, O2 sats 100%
• PE significant for palpable thrill, nl S1, split S2, with 4/6 harsh systolic ejection murmur at LUSB, ejection click, strong pulses 4 extremities, no HSM
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Moss and Adams 8th Edition, 2013
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Pulmonary Valve Stenosis
• Initially described in 1761 by John Baptist Morgagni – thought to be rare
• Common, 8-10% of patients with CHD
• Familial occurrence – 2%
• Cause – Failed cellular migration, cell signaling pathway errors
• Natural history
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Pulmonary Stenosis
• Caused by fused or absent commissures. Leaflet fusion with thickening and valve dysplasia, usually with supravalvar attachments called raphe.
• These valvar abnormalities cause poor leaflet excursion, variable stenosis, and can lead to pulmonary insufficiency
• Myxomatous thick dysplastic leaflets seen in the Noonan’s subtype
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Eheart.org
Revespcardiol.org
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Pulmonary Valve Stenosis
• Normal, trivial, mild, moderate, severe, critical
• Severe and critical require intervention.
• Severe - max instantaneous gradient > 60 mmHg
• Moderate – MIG is 40-60 mmHg
• Mild – MIG is < 40 mmHg
• Gradients affected by sedation, anemia, PDA
• Trivial and mild just get on your radar for progression
• Post-stenotic dilatation of MPA and LPA is common and well tolerated in PS.
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Associated Lesions - Pulmonary Stenosis• Diffuse RV hypertrophy (infundibulum) -dynamic
subvalvar obstruction
• Thickening and dysplasia of tricuspid valve, with regurgitation
• RA dilation / thickening
• PFO vs ASD
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Moss and Adams 8th Edition, 2013
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PS Murmur – other factors
• a hemodynamically significant ASD, increases blood flow across pulmonary valve and makes obstruction appear worse
• PS – while PVR is still high – masks the gradient due to downstream obstruction
• Presence of PDA – competing PBF
• PS – agitation during echocardiogram will increase the gradient
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Pulmonary Valve Stenosis• Why do we care if the RV is pressure loaded with mild
/ moderate PS?
• We do and we don’t• Duration – for a short time is ok• Timing – during infancy• RV features - causes RVH, and decreased compliance of the
RV ensues. Atrial shunting is thus affected. • Crowding on the left ventricle – RV is normally crescent
shaped and wraps around LV.
• Symptoms – none unless untreated severe PS (cyanosis if an ASD, older pts with chest pain, syncope, sudden death with exercise)
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Continuous Doppler 4V^2 = Peak Pressure Gradient
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Nadas’ Pediatric Cardiology
•Peak to peak pressure
gradient in cath lab of ~
60 mmHg (80-20)
•Consistent with
moderate-severe valvar
PS
•Lobby towards
ballooning this valve
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Nadas’ Pediatric Cardiology
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Moss and Adams 6th Edition
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www.hopkinsmedicine.org
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Eheart.org
Revespcardiol.org
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PS Therapy in Evolution
• Balloon pulmonary valvuloplasty if possible, rarely surgical intervention required
• Early cath BPV results were excellent – only 4% of patients required 2nd intervention
• Post-intervention pulmonary insufficiency was thought to be common, mild, and well-tolerated
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Pulm Regurgitation (PI) after BPV
• Moderate PI (PR) post valvuloplasty up to 25%, and 40% in neonates 10 yrs later.
• Mod PI → RV dilation → RV dysfunction and diminished exercise tolerance
• MRI – 10-15 yrs old
• Pulmonary valve replacement
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http://www.utswmedicine.org/
Heartvalvesurgery.com
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Cardioegypt.com
www.miotinternational.com
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Pulmonary Stenosis - summary
• Not a zebra
• Common cardiac disease, seen frequently in the NICU or at newborn visits, with characteristic diagnostic findings
• Managed either expectantly or by cath intervention
• Aggressive balloon dilation is not benign, with pulmonary regurgitation and RV dilation
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Peripheral Pulmonic Stenosis (PPS or PABS)• Newborn murmur, especially common with
premature infants, ejection systolic radiating from the LUSB to axillae and the back
• Cause? – small size of the branch pulmonary arteries following birth and the right angle take-off from the MPA, which becomes less acute with growth.
• LPA PPS after ductal closure very common.
• Murmur should disappear in first 6 months of life.
• Benign – except in Alagille or Williams Syndrome
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Case # 2
• 7 year old twin with years of frequent URIs, worsening stridor, loud breathing, decreased exercise tolerance in comparison to his twin.
• Vitals normal, exam normal
• No respiratory distress noted
• Recent ER and pulmonary visits led to CT
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Vascular Ring
• Anomaly of aortic arch formation leading to complete encirclement of trachea and esophagus by vascular structures
• Two common types consist of 90% of surgical cases• Double aortic arch
• Right aortic arch with aberrant left subclavian artery and diverticulum of Kommerell
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Wikipedia
Cardiologyforyou
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Moss and
Adams 6th
Edition
https://clinicalgate.c
om/congenital-
anomalies-of-the-
thoracic-great-
arteries/
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Nadas’
Pediatric
Cardiology
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Right aortic arch with Aberrant Left Subclavian Artery• 2nd most common vascular ring – includes a retroesophageal
vessel from which the left subclavian artery and left ductus or ligamentum arises.
• Regression of left fourth embryonic arch
• Symptoms – swallowing or respiratory problems (stridor, dyspnea, DOE, cough, resp infections). Most patients are asymptomatic.
• CXR – shows right arch.
• Barium esophagogram – posterior indentation on esophagus
• MRI or CTA – ideal for imaging both airway and vasculature.
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Nadas’
Pediatric
Cardiology
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Double Aortic Arch
• Persistence of embryonic right and left aortic arches with complete encirclement of trachea and esophagus
• Right aortic arch is dominant – 75% of cases. Left arch is either hypoplastic or atretic.
• Same symptoms – stridor, respiratory problems, or swallowing difficulty
• Confirmation not possible by Echo???? (+/-), please proceed with MRI/CTA
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Doubleaorticarch.w
ordpress.com
www.hawaii.edu
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Case # 3
• 6 yo female with referral for murmur – wishy-washy cardiac complaints of intermittent chest pain, fatigue, and palpitations at low level exercise. No syncope, no cyanosis
• PMHx – asthma. No prior surgeries
• Family / Social Hx unremarkable
• Vitals HR-82 RR-24 BP-110/63 RA 139/70 LL
• Exam significant for late systolic click in supine position
• Click moves to mid-systole in standing position, with late systolic regurgitant murmur trailing the click
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Mitral valve prolapse
• Disease of the 1980’s – echo new technology
• Diagnosis made by exam and confirmation by echocardiogram (stretched chordae tendinae)
• Mid-to-late systolic ejection click. https://www.youtube.com/watch?v=sH_KmHIHR70
• Variable regurgitant murmur of MR – usually mild
• Echo shows – non planar Mitral valve with anterior and/or posterior leaflet prolapse with systolic atrial displacement, usually some leaflet thickening
• Echo with valsalva or upright posture should accentuate the prolapse
• Most common cause of MV surgery in developed countries.
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Mayo Clinic web site
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Keyhole Heart Clinic - London
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Mitral valve prolapse
• Consider syndromes – pectus, scoliosis, joint and skin issues
• Consider familial inheritance patterns
• Common in connective tissue disorders• Marfan syndrome
• Ehlers-Danlos syndrome IV (vascular)
• Osteogenesis imperfecta
• Pseudoxanthoma elasticum
• Stickler syndrome
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Mitral valve prolapse murmur
• Systolic click moves closer to S1 with decreases in LV volume (standing or valsalva), usually with more prominent MR murmur
• Systolic click moves later toward S2 with increases in LV volume (squatting), with decrease in MR.
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Moss and Adams 6th Edition
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MVP – Clinical presentation
• Atypical chest pain, palpitations, fatigue, dizziness, syncope, dyspnea, anxiety attacks
• No basis of association of symptoms with MVP – no difference noted vs controls.
• Nonspecific EKG – but atrial (A fib) and ventricular arrhythmias are more common than in the normal population
• Mitral regurgitation is usually trivial to mild – but can progress with age and necessitate valve repair
• Infective Endocarditis – routine dental prophylaxis is not recommended, but MVP valve is at risk of IE.
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MVP – Sports Restriction
• No data exists to demonstrate that strenuous exercise in patients with MVP predisposes to death.
• Common sense and good judgment• Self limit exercise for symptoms (chest pain, fatigue)
• Evaluate left ventricular and left atrial function, mitral valve function, and existing symptoms.
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Questions
• What is the difference between severe and critical valvar PS?
• Why aren’t interventional cath docs as aggressive with valvar PS these days?
• What happens to the MVP click and murmur with standing? Squatting?
• What are the two most common types of vascular ring?
• What is the imaging gold standard for establishing a diagnosis of a vascular ring?
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Case # 4
• 6 week old female with a “murmur”
• No cyanosis, no sweating, increasing fatigue, sleeps a lot, seems pale and mottled with cold extremities
• Febrile illness 2 weeks ago with diarrhea
• Hyperemesis and preeclampsia during pregnancy
• Family history – paternal hypothyroidism
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Physical Exam
• HR 153, RR 52, BP 76/49 LL
• Nl resp effort, clear lungs, no cyanosis
• PMI displaced laterally, increased precordial activity, gallop present, 1/6 systolic regurgitant murmur @ apex, pulses normal
• Obvious pallor, liver edge down 2 cm
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Dilated Cardiomyopathy
• Dilation and impaired systolic function of left or both ventricles – “Hefty bag”
• Most common of the cardiomyopathies (CM) –50%. Incidence is 0.58 cases per 100,000 kids
• Causes – Infectious, Familial, Mitochondrial, Metabolic, Arrhythmic, Toxic, Inflammatory, and Idiopathic
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Inflammatory
• Viral myocarditis leads to abnormal immunologic response (autoimmune), causing tissue destruction. “Burnt-out Myocarditis” – 30-40% of cases
• Coxsackie B (enteroviruses), adenovirus, herpes simplex, cytomegalovirus
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Clinical Presentation
• Older kids – exercise intolerance, DOE, tachycardia, palpitations, chest pain, syncope or near syncope, occasionally with cardiovascular collapse and sudden death
• Younger kids – rarely present with adult findings of peripheral edema and postural nocturnal dyspnea
• Infants – respiratory distress, abdominal distension, poor feeding, failure to thrive
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Physical Findings - DCM
• Tachypnea, tachycardia, weak pulses, narrow pulse pressure, hypotension, cool extremities, poor capillary refill, wheezing, hepatomegaly
• Laterally and widely displaced cardiac impulse, gallop (S3), Mitral Regurgitation murmur
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Diagnostic Tests in DCM
• CXR – cardiomegaly, pulmonary venous congestion, pulmonary edema, effusions, and atelectasis
• EKG – Sinus Tachy, ST-T wave changes, LVH, RVH
• Holter - Afib / Aflutter, PVCs, Ventricular tachycardia – 50% of patients at diagnosis
• Echo – LV dilation, decreased systolic performance, pericardial effusions, thrombi, Mitral regurgitation
• Cath – biopsy and pre-transplant hemodynamics
• Treatment – supportive care, Lasix, Digoxin, Aldactone, milrinone, carvedilol, ASA, prayers
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Questions???
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IE Prophylaxis
• Summary from “Prevention of Infective Endocarditis. Guidelines from American Heart Association.” May 8, 2007.
• New recommendations include prophylaxis only for dental prophylaxis on patients with• Prosthetic cardiac valve
• Previous infective endocarditis
• Congenital heart disease **
• Cardiac transplant recipients who develop valvulopathy
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IE in Congenital Heart Disease
• IE prophylaxis recommended in CHD with• Unrepaired cyanotic heart disease, including palliative
shunts and conduits
• Completely repaired congenital heart defect with prosthetic material or device during first 6 months following procedure (while endothelialization occurs)
• Repaired CHD with residual defects at site (VSD patch leak – prevents endothelialization)
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IE
• Dental procedures cause transient bacteremia• Tooth extraction (10-100%)• Periodontal surgery (36-88%)• Scaling and root planing (8-80%)• Teeth cleaning (up to 40%)
• Routine daily dental procedures also cause transient bacteremia• Tooth brushing and Flossing (20-68%)• Wooden toothpicks (20-40%)• Chewing food (7-51%)
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IE
• The frequency of bacteremia and therefore risk of endocarditis is far greater due to routine daily activities
• Vast majority of IE cases had no recent dental procedure 2 weeks before onset of symptoms
• Current emphasis is for maintenance of good oral hygiene and eradication of dental disease in effort to decrease incidence of IE.
• Risk of antibiotic adverse events may exceed benefit of prophylaxis in many patients
• Additional research is encouraged to substantiate future recommendations.
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Venous Hum
• Best auscultated over the right or left upper chest to low neck area– continuous
• Disappear with changes in head position or compression of the jugular vein
• Vary with respiration
• Best heard with patient upright
• Turbulence of venous flow IJ or EJ