Coarctation of Aorta

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Case Presentation:

Case Presentation By Obay Al- Rawi

HISTORY:

HISTORY Abdullah Mohammed Saad , a 13 year-old male patient from Raima , Al- Salafiyah , studies in the 7 th level of primary school. History was taken from the patient, his mother and sister. He was admitted to our hospital on January the 16 th with a 4 day history of headache. 2

Slide 3:

The condition started 4 days before admission as sudden onset continuous headache described by the patient as “heaviness in the head”, not relieved by analgesia, associated with blurring of vision, dizziness, fever, anorexia, nausea and repeated attacks of projectile greenish vomiting. 3

Slide 4:

There is a history of poor weight gain in the last 6 months. No history of convulsion, weakness, altered consciousness, or decreased hearing. No other history of significance in the review of other systems. No history of previous admission or blood transfusion. There is a history of contact with a bat 2 months ago with no documented bite. 4

Slide 5:

He is on home food, and he received vaccinations until 9 months of age. There is no consanguinity, and he is the 9 th child in the family with 10 other siblings, 8 are alive and healthy, and two died at infancy due to fever and dehydration. Besides, there is one abortion. No history of chronic illnesses in the family. Good socioeconomic history, with a well ventilated house with electricity and water supply. 5

Slide 6:

The patient was admitted to PER for 3 days, then shifted to isolation as a case of CNS infection where he stayed for a full one month, his condition fluctuated there with alteration of consciousness, increased irritability, poor sleep, persistent fever, spasticity, and an erythematous skin rash, with progressive improvement in most of the symptoms over time of admission. 6

Physical Examination:

Physical Examination Looks ill, conscious, irritable, afebrile , no pallor, no jaundice. Body weight: 26 Kg PR: 60 bpm BP: 160/100 mmHg Chest: clear Heart: S1+S2+systolic murmur Abdomen: soft Neck stiffness with symmetrical reactive pupils No focal neurological deficit or weakness 7

Important events during Admission Clinical:

Important events during Admission Clinical Persistence of fever and hypertension until few days before discharge UMNL manifested by hypertonia with sustained ankle clonus and hyperreflexia Irritability and abnormal behaviour Funduscopy on 26/1 : papilledema 8

Important events during Admission Investigations:

Important events during Admission Investigations CBC: Hb : 16 WBC: 14.1 N: 82% L: 10% M: 7% E: 1% platelets: 339 Ten days later: CBC: Hb : 13.2 WBC: 10.2 N: 71% L: 9% M: 18% E: 2% platelets: 342 Normal RFT, S. electrolytes and urine analyses CRP, Brucella and Widal – ve 9

Important events during Admission Investigations:

Important events during Admission Investigations CSF: “5 days from onset of symptoms” Bloody, protein: 18 sugar: 53 WBC: 18 RBC: 20,000 Three weeks later: CSF: clear, completely normal Urine culture and blood culture are both + ve for Staph. aureus 10

Important events during Admission Brain imaging:

Important events during Admission Brain imaging 17/1 Signs of meningoencephalitis by mild brain edema and increased ICP with prominent ventricular system. 25/1 Deep white matter hypodensities more obvious at the frontoparietal region due to edema mostly post meningoencephalitis associated with increased ICP evident by effacement of brain sulci . Prominence of whole ventricular system. No intracranial hemorrhage. 11

Important events during Admission Brain imaging:

Important events during Admission Brain imaging 6/2 Ill defined hypodensities ( cerebritis ), with gyral hyperdensities at the frontoparietal region bilaterally representing cortical hemorrhage as complication of acute meningoencephalitis 14/2 (MRI) Sign of acute encephalopathy affecting mainly the frontal lobe to frontoparietal region Rt.>Lt. with secondary ischemic defect. No signs of increased intracranial pressure. 12

Important events during Admission Treatment:

Important events during Admission Treatment A 2-week course of vancomycin and ceftriaxone at antimeningeal dose. A 10-day course of acyclovir A 7-day course of Quinine A 7-day course of Amikacin Antihypertensives ( Lasix , Captopril and nifedipine ) Mannitol , dexamethasone , phenobarbitone and acetazolamide . Anti-Rabies Ig dose. 13

Final Diagnosis:

Final Diagnosis Coarctation of Aorta + Meningoencephalitis 14

Coarctation of Aorta:

Coarctation of Aorta 15

Definition:

Definition 16

prevalence:

prevalence Coarctation of the aorta is a common malformation. Accounts for 6 to 8 percent of all congenital heart defects. Two to five times more frequently in males than females. Most cases occur sporadically and familial recurrence was thought to be rare. 17

Associations:

Associations The prevalence of coarctation is increased in certain disorders, such as Turner syndrome. There is a frequent association of coarctation of the aorta with a bicuspid aortic valve. Ventricular septal defect. Patent ductus arteriosus (PDA). Aortic stenosis ( valvular , subvalvular , or supravalvular ). Parachute mitral stenosis . The most important non-cardiac abnormality is intracerebral aneurysm. 18

Presentation:

Presentation Symptomatic infants: Congestive heart failure and shock in neonatal period. The patient will be pale, irritable, dyspneic , with tachycardia, tachypnea , diaphoresis and hepatomegaly . Differential diagnosis with right to left ductal shunt. 19

Presentation:

Presentation 20

Presentation:

Presentation 2. Asymptomatic children: Arterial hypertension. Heart murmur. Chest pain and legs claudication with exercise. Frequent headaches. Cold extremities. 21

Presentation:

Presentation The hallmark of COA is a difference in systolic blood pressure between the upper and lower extremities; the diastolic blood pressures are typically similar. The classic findings are hypertension in the upper extremities, diminished or delayed femoral pulses (brachial-femoral delay), and low or unobtainable arterial blood pressure in the lower extremities 22

Diagnosis (ECG):

Diagnosis ( ECG) May be normal. Infants with severe coarctation display pure right ventricular hypertrophy. In older children and adults, the ECG may show left ventricular hypertrophy, with increased voltage and ST and T wave changes in the left precordial leads 23

Diagnosis (CXR):

Diagnosis (CXR) 24

Diagnosis (CXR):

Diagnosis (CXR) 25

Diagnosis (CXR):

Diagnosis (CXR) 26

Diagnosis (Echocardiography):

Diagnosis (Echocardiography) 27

Diagnosis ( MRI):

Diagnosis ( MRI) 28

Diagnosis (Cardiac catheterization):

Diagnosis (Cardiac catheterization) 29

Treatment:

Treatment MEDICAL For the sick neonate who presents with severe congestive heart failure or shock ( ductal -dependent left-sided obstructive lesion): Prostaglandin infusion: 0.05 µg/kg per minute (anticipating adverse effects including apnea) Inotropic support Diuretics for pulmonary venous hypertension or pulmonary edema Surgical intervention should follow as soon as possible. 30

Treatment:

Treatment SURGICAL Infancy Surgical repair of severe coarctation and coarctation associated with intracardiac anomalies The surgical mortality rate for infants with coarctation and a large VSD ranges from 5% to 15% and is higher for children with more complex intracardiac anomalies. Childhood Elective coarctation repair between ages 18 months to 3 years in asymptomatic children without severe upper extremity hypertension. Later repair is associated with an increased risk of sustained hypertension and other late complications. 31

Treatment:

Treatment Types of Surgical Repair End-to-end anastomosis Subclavian flap aortoplasty Prosthetic patch aortoplasty Bypass graft Nonsurgical Option Percutaneous balloon angioplasty of native coarctation in infants and children with or without stent is pursued in some centers. 32

Prognosis :

Prognosis The long-term prognosis following repair of coarctation may be adversely affected by: Systemic arterial hypertension. Recoarctation . Aortic aneurysms and rupture. 33

Prognosis:

Prognosis Untreated coarctation has a poor natural history with the onset of congestive heart failure, especially in those patients with other intracardiac malformations with a mortality rate about 88%. Untreated coarctation in asymptomatic patients with mean survival age of 34 years. 34

Prognosis:

Prognosis Most common causes of death are: CHF 26%. Aortic rupture 21%. Bacterial endocarditis 18%. Intracranial hemorrhage 12%. 35

What Happened???:

What Happened??? 36

Scenario 1 Meningoencephalitis (Viral):

Scenario 1 Meningoencephalitis (Viral) With Fever, headache, projectile vomiting and meningismus . Leukocytosis . Brain imaging findings. Focal cerebritis . UMNL. 37

Scenario 1 Meningoencephalitis (Viral):

Scenario 1 Meningoencephalitis (Viral) Against Not related to the primary illness. Normal CSF. 38

Scenario 2 Rabies:

Scenario 2 Rabies With History of contact with a bat 2months before admission. Encephalopathy. 39

Scenario 2 Rabies:

Scenario 2 Rabies Against : No documented bite. No hydrophobia or aerophobia. The patient is still alive. 40

Scenario 3 Hypertensive encephalopathy:

Scenario 3 Hypertensive encephalopathy With Headache and vomiting. Hypertension. Encephalopathy. 41

Scenario 3 Hypertensive encephalopathy:

Scenario 3 Hypertensive encephalopathy Against Fever. Brain imaging findings. CNS sequelae . 42

Scenario 4 Aneurysmal Subarachnoid Hemorrhage:

Scenario 4 Aneurysmal Subarachnoid Hemorrhage With Sudden headache, vomiting and meningismus . Age at presentation (10-30 years). Pink bloody CSF. Increased ICP with prominent ventricular system. Aseptic meningitis. Vasospasm with delayed cerebral infarction. 43

Scenario 4 Aneurysmal Subarachnoid Hemorrhage:

Scenario 4 Aneurysmal Subarachnoid Hemorrhage Against : Fever. Brain CT scan – ve for Hemorrhage. 44

Scenario 5 Bacterial endocarditis equivalent (Endarteritis):

Scenario 5 Bacterial endocarditis equivalent (Endarteritis) With Fever, anorexia and weight loss. Predisposing factor (COA). Positive blood culture. Focal neurological deficit (aseptic meningitis). Embolic phenomena (CVA). 45

Scenario 5 Bacterial endocarditis equivalent (Endarteritis):

Scenario 5 Bacterial endocarditis equivalent (Endarteritis) Against Should have more than one + ve blood culture. Negative Echo study for endarteritis. 46

Recommendations :

Recommendations Every patient with systemic arterial hypertension should have the brachial and femoral pulses palpated simultaneously to assess timing and amplitude to search for the "brachial-femoral delay" of significant aortic coarctation . Supine bilateral arm (brachial artery) blood pressures and prone right or left supine leg ( popliteal ) blood pressures should be measured to search for differential pressure. 47

Thank You:

Thank You 48

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