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coarcatation of the aorta DXR
- 1. Case Presentation Pediatrics week 4 Preceptor: Dr. Geneviene Tupas
- 2. Patient identifying data • D. E • 12 years old • Male • Single • Latino • Source of history : mother • Reliability: 95%
- 4. History of present illness 4 • At 2 weeks old, the patient had first checkup after birth, where doctor noted a murmur. But it was told fine • At 2 years old, the patient consulted cardiologist, where he also noted a murmur, which was also told fine at that time. • 1 year PTC, patient started experiencing fatigue • He used to be rested enough with 8 hours of sleep, now requires 11 hours of sleep, still feels tiered.
- 5. History of present illness 5 • When asked for severity, mother stated: “progressively concerning, his performance at sports and studies were also affected by this”. • He usually have leg cramps while exercising, which resolves through rest • Headache 2 to 3 times a week, mostly relieved by Tylenol.
- 6. Past medical history • Experienced ear infections between 6 to 12 months of age • Had stitches for cut on his chin when he was 4 years old • Left radius fractured at 7 years old while falling of a slide
- 7. Birth History • Born in San Francisco, CA • Full term Delivery • Birth weight: 7 lbs • Height at birth: 14.5 inches • G2P2
- 8. Nutrition & Feeding history • Well balanced diet • Mother is aware of feeding • Meal contains protein, fruit or a vegetable • Drinks 1-2 glasses of milk everyday • Consume beans upto 5 times per week
- 9. Immunization history • All necessary vaccines are given up to age • MMR, Tdap, Meningococcal, Hepa B were given • Planning to get HPV vaccine
- 10. Growth & Development • All milestones Are appropriate to age • He always appear to be on smaller side • Walking by 1 year old • Talking by 3 years old • Toilet trained by 21 2 years old
- 11. Family History
- 12. Personal & Social History • Patients' parents drink beer or wine occasionally at a family social event • He attends middle school till afternoon and goes to soccer or football practice after school, but he is not playing hard due to his fatigue and leg cramps • He completes his homework after dinner but due to his fatigue he sleeps while doing homework
- 13. Personal & Social History • He has an elder brother who is 15 years old • He is allergic to cats • His mother is a homemaker, his father is an assistant manager in a Grocery store.
- 14. HEADSSS 14 Home- patient family is well supportive Education- he had school attendance & good grades Activity- he plays and enjoy his life with friends Drugs- he isn’t smoke or drink Suicidality- he doesn’t have suicidal thoughts Sex- he wasn’t into sexual relationships Safety- he feels safe at home
- 15. Review of systems • General: fatigue, weight change(-) • Skin: Night sweats(-), Rash(-) • Head: dizzines (-), Headache(-) • Eyes: Eye dryness (-), Redness (-) , itchy(-) • Ears: Tinnitus (-), Vertigo (-) • Nose and sinuses: Rhinorrhea (-), stuffiness (-),runny nose(-)
- 16. Review of systems • Throat: Dysphagia (-), Sore throat (-) • Breast: Pain (-), Lumps (-) • Respiratory: Orthopnea (-), nocturnal dyspnea (-) , cough(-) • Cardiovascular: Palpitation (-), Edema (-) • Gastrointestinal: Constipation (-), Diarrhea (-)
- 17. Review of systems • Genitourinary: Dysuria (-), Discharge (-) • Musculoskeletal: Weakness (-), Pain (-) • Neurological: Balance difficulty (-), Double vision (-) • Hematologic: Bleeding (-), Anemia (-) • Endocrine: Excessive sweating(-), Excessive thirst (-)
- 19. P E Findings 20 General : well nourished & active Anthropometric measurements : Length – 150 cm Weight – 42kgs BMI - 18.7kg/m2 Vital signs: B.P - 130/86mmhg T - 98.6º F R.R - 16bpm P.R - 100bpm O2 saturation - 97%
- 20. Growth Charts 21 Interpretation: Weight: 42 kgs Height: 150 cm BMI: 18.7 kg/m2 Z score: 0 to 1 Normal
- 21. Skin & HEENT: Normal • SKIN: warm, dry with good turgor. NO abnormal pigmentation, bleeding, rash or other lesions. • HEENT: • SKULL: smooth, symmetrical with no evidence of deformity, tenderness or masses. • FACE: symmetrical with no evidence of drooping or paralysis and rashes. • EYES: symmetrical in size shape and color. NO evidence of discharge, redness or swelling. Pupils are equal in size, constrict and dilate appropriately in response to light.. Visual acuity 20/20 • EARS: symmetrical with no evidence of deformity, discharge and swelling. Ear canals clear free of blockages. Ear drum intact and translucent. • NOSE: septum midline, no nasal deviation, devoid of polyps. No evidence of discharge or bleeding. • MOUTH: tongue and uvula is in midline. Free of lesions or sores with healthy gums and teeth. No enlarged o inflamed tonsils. • NECK: supple and mobile with no evidence of masses or stiffness. non palpable thyroid gland, lymph nodes small and non tender.
- 22. Chest & Lungs: Normal • INSPECTION: skin is smooth without lesions. Chest symmetric, trachea is midline . Ratio of ap to lateral diameter is 1:2. Respiratory movements are full symmetric without retractions. Breathing regular without use of accessory muscles. • PALPATION: no evidence of tenderness and masses. vocal fremitus is symmetric and moderate. • AUSCULTATION: Breathe sounds readily heard throughout the lungs are symmetric, low pitch soft intensity.
- 23. Cardiovascular • Inspection: Adynamic pericardium, No masses, no lesions were present • Palpation: PMI at 5th ICS left midclavicular line. Systolic thrill over left upper sternal border • Auscultation: Regular cardiac rate and rhythm. Grade IV/VI systolic murmur heard throughout systole and radiate to the back in mid scapular area. Murmur was heard louder at the base of heart compared to aortic and pulmonic
- 24. Abdomen : Normal • INSPECTION: symmetric, flat shaped without lesions. • AUSCULTATION: clicks and gurgles are heard 10-15 times per min. • Percussion: tympanic sounds noted. • PALPATION: no tenderness and pain noted.
- 25. EXTREMITIES • capillary refill time 2-3 sec, no edema. Slightly pale palmar area. • No acrocyanosis and clubbing of fingers. • Blood pressure was taken at all four extremities: • Right Upper arm : 135/98 mmHg • Left upper arm: 130/86 mmHg • Right thigh: 92/60 mmHg • Left Thigh: 84/60 mmHg • Muscles in thighs are not well developed compared to the upper extremities.
- 26. Neurosensory: Normal • CN-I(Olfactory) - sensation of smell is intact • CN-II(Vision)- visual acuity is good and it is 20/20 • CN-III,IV,VI-(extraocular movements)- movement of eye is symmetrical and tracking the objects clearly • CN-V(Motor)-chewing the food without difficulty • CN-VII(Facial)- no asymmetry in facial expression • CN-VIII(Acoustic)- whisper, weber, rinne tests are positive • CN-IX,X- gag reflex is intact • CN-XI(Spinal accessory)-Shoulders are symmetrical and there is a good range if motion • CN-XII(Hypoglossal)-Tip of the tongue is at midline, no spasticity in tongue movements • Sensory : sensation to touch, pain, hearing are all intact.
- 27. Laboratory
- 28. CBC w/Diff: Normal • Wbc-12.0 K/Mm3 (High) • Rbc-4.6 Cube • Hbg: 12.5 G/Dl • Hct: 36.2 % • MCH: 26 Pg • MCHC: 33 G/ Dl • MCV: 80 Fl • Platelets: 315 Ku/ L • Total Lymphocytes: 58% WBC Count (HIGH) • Neutrophils, Band: 3.0% WBC • Neutrophils, Segmented: 31.4% WBC • Eosinophils :2.5% WBC • Basophils: 0.5% WBC • Monocytes: 4.6% WBC
- 29. Chest X Ray • There is an evidence of scalloping of the mediastinum most notable along the left mediastinal border. • Figure 3 sign (double bulge) can be observed at aortic arch • There are no mediastinal masses noted. • There are no fractures or abnormal calcifications of the ribs noted.
- 30. ECHOCARDIOGRAM • There is mild left ventricular hypertrophy with the inter-ventricular septum and left ventricular free walls measuring 1.1 cm. • Doppler and color doppler interrogation of the valves, ascending aorta and aortic arch demonstrate a coarctation of the aorta present at the juxta-ligamental position • The peak-to-peak pressure gradient across the coarctation is estimated 42 mm • The ascending aorta is noted to be slightly enlarged
- 31. ECG • There is a evidence of mild left ventricular hypertrophy. • No rhythm disturbances are noted
- 32. Left ventricular hypertrophy secondary to Coarctation of the Aorta Diagnosis
- 33. Differentials Fatigue Cardiac Neurologic infectious Others Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Depression Tuberculosis Endocarditis Hepatitis Group A Strept Acute malnutrition Chronic Fatigue syndrome Systolic murmur & left ventricular hypertrophy were observed on PE
- 34. Differentials Fatigue Cardiac Neurologic infectious Others Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Depression Tuberculosis Endocarditis Hepatitis Acute malnutrition Chronic Fatigue syndrome Systolic murmur observed on PE Cardiac Neurologic infectious Others Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Depression Tuberculosis Endocarditis Group A Strept Acute malnutrition Chronic Fatigue syndrome
- 35. Differentials Fatigue Cardiac Neurologic infectious Others Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Depression Tuberculosis Endocarditis Hepatitis Acute malnutrition Chronic Fatigue syndrome Systolic murmur were observed on PE Cardiac infectious Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Tuberculosis Endocarditis Group A Strept No cough, sputum, fever
- 36. Differentials Fatigue Cardiac Neurologic infectious Others Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Depression Tuberculosis Endocarditis Hepatitis Acute malnutrition Chronic Fatigue syndrome Systolic murmur observed on PE Cardiac infectious Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Tuberculosis Endocarditis Hepatitis Group A Strept No cough, sputum, fever Cardiac infectious Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Tuberculosis Endocarditis Group A Strept
- 37. Differentials Fatigue Cardiac Neurologic infectious Others Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Depression Tuberculosis Endocarditis Hepatitis Acute malnutrition Chronic Fatigue syndrome Systolic murmur observed on PE Cardiac infectious Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Tuberculosis Endocarditis Hepatitis Group A Strept No cough, sputum, fever Cardiac infectious Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Tuberculosis Endocarditis Group A Strept No cough, No sputum No fever No chest pain No rales or abnormal breath sounds observed No neurologic deficit No lymphadenopathy
- 38. Differentials Fatigue Cardiac Neurologic infectious Others Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Depression Tuberculosis Endocarditis Hepatitis Acute malnutrition Chronic Fatigue syndrome Systolic murmur observed on PE Cardiac infectious Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Tuberculosis Endocarditis Hepatitis Group A Strept No cough, sputum, fever Cardiac infectious Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Tuberculosis Endocarditis Group A Strept No fever No chills Echocardiograph shows no signs of endocarditis and vegetation
- 39. Differentials Fatigue Cardiac Neurologic infectious Others Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Depression Tuberculosis Endocarditis Hepatitis Acute malnutrition Chronic Fatigue syndrome Systolic murmur observed on PE Cardiac infectious Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Tuberculosis Endocarditis Hepatitis Group A Strept No cough, sputum, fever Cardiac infectious Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Tuberculosis Endocarditis Group A Strept Age of the patient No fever No strept throat
- 40. Differentials Fatigue Cardiac Neurologic infectious Others Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Depression Tuberculosis Endocarditis Hepatitis Acute malnutrition Chronic Fatigue syndrome Systolic murmur observed on PE Cardiac infectious Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Tuberculosis Endocarditis Hepatitis Group A Strept No cough, sputum, fever Cardiac • Myocarditis • Mitral Valve Prolapse • Aortic Stenosis • Dilated Cardiomyopathy • Coarcataion of Aorta Headaches, exercise intolerance, left ventricular Hypertrophy
- 41. Differentials Fatigue Cardiac Neurologic infectious Others Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Depression Tuberculosis Endocarditis Hepatitis Acute malnutrition Chronic Fatigue syndrome Systolic murmur observed on PE Cardiac infectious Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Tuberculosis Endocarditis Hepatitis Group A Strept No cough, sputum, fever Cardiac • Myocarditis • Mitral Valve Prolapse • Aortic Stenosis • Dilated Cardiomyopathy • Coarcataion of Aorta Headaches, exercise intolerance, left ventricular Hypertrophy Cardiac • Myocarditis • Mitral Valve Prolapse • Aortic Stenosis • Dilated Cardiomyopathy • Coarcataion of Aorta CXR & echocardiogram were done
- 42. Differentials Fatigue Cardiac Neurologic infectious Others Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Depression Tuberculosis Endocarditis Hepatitis Acute malnutrition Chronic Fatigue syndrome Systolic murmur observed on PE Cardiac infectious Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Tuberculosis Endocarditis Hepatitis Group A Strept No cough, sputum, fever Cardiac • Myocarditis • Mitral Valve Prolapse • Aortic Stenosis • Dilated Cardiomyopathy • Coarcataion of Aorta Headaches, exercise intolerance, left ventricular Hypertrophy Cardiac • Myocarditis • Mitral Valve Prolapse • Aortic Stenosis • Dilated Cardiomyopathy • Coarcataion of Aorta CXR & Echocardiogram were done • Myocarditis • Mitral Valve Prolapse • Aortic Stenosis • Dilated Cardiomyopathy • Coarcataion of Aorta Scalloping of mediastinum. Coarctation of the aorta at juxta ligamental position
- 43. Differentials Fatigue Cardiac Neurologic infectious Others Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Depression Tuberculosis Endocarditis Hepatitis Acute malnutrition Chronic Fatigue syndrome Systolic murmur observed on PE Cardiac infectious Myocarditis Mitral Valve Prolapse Aortic Stenosis Dilated Cardiomyopathy Coarcataion of Aorta Tuberculosis Endocarditis Hepatitis Group A Strept No cough, sputum, fever Cardiac • Myocarditis • Mitral Valve Prolapse • Aortic Stenosis • Dilated Cardiomyopathy • Coarcataion of Aorta Headaches, exercise intolerance, left ventricular Hypertrophy Cardiac • Myocarditis • Mitral Valve Prolapse • Aortic Stenosis • Dilated Cardiomyopathy • Coarcataion of Aorta CXR & Echocardiogram were done • Myocarditis • Mitral Valve Prolapse • Aortic Stenosis • Dilated Cardiomyopathy • Coarcataion of Aorta Scalloping of mediastinum. Coarctation of the aorta at juxta ligamental position Final Diagnosis: Coarcatation of the Aorta
- 44. Discussion
- 45. • Coarctation = Narrowing (constriction) • most commonly occurs just beyond the left subclavian artery. INTRODUCTION
- 46. EPIDEMIOLOGY • Found in 6% to 8% of patients with congenital heart disease • Prevalence of coarctation of aorta is high in turner syndrome (45.X) is high as 15-20% • Male >female 2:1 • Bicuspid aortic valve is commonly associated with coarctation of the aorta. • Offspring and other first-degree relatives diagnosed with an obstructive left-sided cardiac lesion are at ten times the risk of coarctation and other cardiac lesions.
- 47. ETIOLOGY • The cause of coarctation of the aorta is unclear. The condition is generally a heart problem present at birth (congenital heart defect). The ductal tissue is thought to cause constriction in the adjacent region of the aorta. This narrows the lumen of the aorta. • Rarely, coarctation of the aorta develops later in life. Conditions or events that can narrow the aorta and cause this condition include: • Traumatic injury • Severe hardening of the arteries (atherosclerosis) • Inflamed arteries (Takayasu arteritis)
- 48. PATHOGENESIS • Hemodynamic theory states that reduced anterograde intrauterine blood flow to the fetal arch leads to its underdevelopment • Ductal hypothesis postulates the migration of ductal tissue into the wall of the fetal thoracic aorta • NOTCH1 gene, which plays an important role in cardiovascular development, and several other genes have been implicated in the etiology of CoA • Mechanical models indicate that blood flow abnormalities, defective endothelial cell migration, and excessive deposition of aortic duct tissue at the aortic isthmus can lead to coarctation
- 49. • Coarctation of the aorta can occur as a discrete juxtaductal obstruction or as tubular hypoplasia • It is postulated that coarctation may be initiated in fetal life by the presence of a cardiac abnormality that results in • decreased blood flow anterograde through the aortic valve (e.g., bicuspidaortic valve, VSD). • Alternatively, by abnormal extension of contractile ductal tissue into the aortic wall. PATHOPHYSIOLOGY
- 54. CLINICAL MANIFESTATIONS -The age of presentation and manifestations depend on the severity of narrowing, relationship with arch vessels, and collateral vessel formation -Neonates with ductal dependent or “critical coarctation” often present with heart failure, acidosis, and shock following closure of the ductus arteriosus. -Clinical presentation in children and adolescents is typically through lower extremity weakness/pain and exertional dyspnea. -The radial and femoral pulses should always be palpated simultaneously for the presence of a radial-femoral delay. -Upper extremity hypertension and lower blood pressure in the lower extremities with delayed femoral pulses
- 55. Symptoms and complications secondary to hypertension include ● Headaches ● Epistaxis ● Exercise intolerance ● Angina ● Shortness of breath due to left ventricular dysfunction ● Heart failure ● Ruptured cerebral artery aneurysms
- 56. Cardiac auscultation may demonstrate a harsh systolic murmur in the left sternal border with radiation to the inter- scapular region in the back. In the suprasternal notch, an associated thrill may also be palpable. Occasionally, a left ventricular lift can be observed of there is left ventricular pressure or volume overload.
- 59. BEFORE SURGERY AND AFTER SURGERY
- 60. Medications • Propranolol (20mg-40mg ,BID, po) for hypertension (before surgery and after surgery) • Enalapril (0.05 mg,three times a day,IV)) for to decrease after load( before surgery)
- 61. Prognosis • Coarctation of the aorta can be cured with surgery. Symptoms quickly get better after surgery. • However, there is an increased risk for death due to heart problems among those who have had their aorta repaired. Lifelong follow-up with a cardiologist is encouraged • Early mortality was 4% in patients with isolated coarctation. Actuarial survival rates were 90%, 88% and 88% at 5, 10 and 17 years of follow-up, respectively. Complex coarctation was associated with decreased survival (p = 0,007)
- 62. Follow Up • Long term follow up is needed to monitor and to evaluate the patient condition and also there is a high chances of refractory hypertension and restenosis, further complications after surgery. • Initially the follow up should be for every month and later it should be for every two months.
- 63. Thank You