Age: 26
Gender: Male
Occupation: Engineer
Working diagnosis: Subarachnoid hemorrhage, suspected aortic coarctation
HISTORY
The patient was well throughout childhood and adolescence, without physical limitation or any health problems.
Three weeks ago he suddenly developed a severe headache associated with transient loss of consciousness. He had neck stiffness and was slightly confused. He complained of severely impaired vision bilaterally as well as impairment of his visual fields. Examination at the time showed bilateral subretinal hemorrhages. A CT scan of the brain showed diffuse subarachnoid hemorrhage.
A cerebral angiogram via the right femoral route was attempted but failed because the catheter could not be advanced past the thoracic descending aorta. Instead, the study was performed via the right brachial approach. The angiogram revealed an anterior communicating artery aneurysm.
The patient underwent right frontal craniotomy and successful clipping of the aneurysm. Postoperatively he had a left hemiparesis, which resolved over the following days.
He had smoked for 10 years and occasionally drank alcohol.
Comments: Twelve percent of patients presenting with sudden-onset headache with a normal neurological examination, and up to 25% of those with an abnormal examination, have a subarachnoid hemorrhage. The mortality rate for a subarachnoid hemorrhage is 50%.
Risk factors for intracranial aneurysm include advanced age (>50 years), female gender, cigarette smoking, cocaine use, hypertension, head trauma, and inherited factors such as autosomal dominant polycystic kidney disease, Ehlers-Danlos syndrome, hereditary hemorrhagic telangiectasia, pseudoxanthoma elasticum, alpha 1 -antitrypsin deficiency, aortic coarctation, Klinefelter’s syndrome, Noonan syndrome, and alpha-glucosidase deficiency.
The first-line investigation for a suspected subarachnoid hemorrhage is high-resolution CT scan of the brain. Sensitivity is 98% if done within the first 12 hours, and lower if the study is done later. Digital subtraction angiography is the gold standard for identifying the source of hemorrhage.
Treatment of a ruptured intracranial aneurysm with open craniotomy and clipping has a 2.6% mortality and 10.9% morbidity. Endovascular treatment for such aneurysms is still evolving.
The history of an intracranial aneurysm and the failure of cerebral angiogram via the right femoral route should raise strong suspicions for coarctation of the aorta (CoA). Ten percent of the patients presenting to the emergency department with ruptured intracranial aneurysm have undiagnosed coarctation.
CURRENT SYMPTOMS
Prior to his cerebral hemorrhage the patient denied any symptomatology.
The patient was recovering from his operation and regaining function on his left side. Vision was still incomplete.
His blood pressure was low in the immediate postoperative period but gradually became severely elevated and controlled with atenolol and amlodipine.
NYHA class: I (prior to these events)
Comments: Most patients with suspected CoA are asymptomatic. However, in some cases, a careful history may reveal exercise limitations compared to peers, particularly involving lower body strength and stamina.
The diagnosis of coarctation is made in most cases because of the discovery of high blood pressure.
If symptoms are present, they can be attributed either to upper body hypertension, such as headache, nose bleeding, dizziness, and tinnitus, or to lower body hypoperfusion, including abdominal angina and exertional leg fatigue. True leg claudication may suggest the presence of abdominal coarctation.
Very occasionally patients come to medical attention with symptoms of LV failure, aortic dissection, or intracranial hemorrhage, as with our patient.
PHYSICAL EXAMINATION
BP 134/76 mm Hg (right arm), 97/46 (right leg); HR 60 bpm, oxygen saturation 99% on room air
Height 177 cm, weight 73 kg, BSA 1.89 m 2
Surgical scars: Right frontal craniotomy
Neck veins: JVP is not elevated.
Lungs/chest: Chest was clear.
Heart: The rhythm was regular. The apex was not displaced. The right and left radial pulses were strong, but his femoral pulses were relatively weak and there was an appreciable radiofemoral delay. The apex was not displaced. He had a normal first and split second sound with an early systolic click and a grade 2–3/6 ejection systolic heart murmur best heard at the back.
Abdomen: Soft, nontender, nonpalpable spleen and liver, normal bowel sounds
Extremities: No clubbing of the fingers or toes, and no edema
Neurological examination: The left hemiparesis had now completely resolved.
Ophthalmologic assessment: Complete right hemianopsia. Normal visual acuity and cranial nerve function.
Comments: Most adults with CoA are asymptomatic, and the diagnosis is made when systemic arterial hypertension is observed in the arm(s) with diminished femoral pulses during routine physical examination.
On physical examination the cardinal sign of CoA is differential blood pressure and pulses between upper and lower extremities. The femoral pulse is weak and delayed. A systolic thrill may be palpable in the suprasternal notch, and LV enlargement may be noted. A systolic ejection murmur can be identified along the left sternal border and in the back, particularly over the coarctation. A continuous murmur caused by flow through the collateral vessels may be heard in the back. A systolic ejection click, due to bicuspid aortic valve, is very common in patients with aortic coarctation.
LABORATORY DATA
| Hemoglobin | 14.7 g/dL (13.0–17.0) |
| Hematocrit/PCV | 43% (41–51) |
| MCV | 91 fL (83–99) |
| Platelet count | 194 × 10 9 /L (150–400) |
| Sodium | 138 mmol/L (134–145) |
| Potassium | 4.3 mmol/L (3.5–5.2) |
| Creatinine | 0.7 mg/dL (0.6–1.2) |
| Blood urea nitrogen | 5.1 mmol/L (2.5–6.5) |
Comments: No abnormal values present.
ELECTROCARDIOGRAM
FINDINGS
Heart rate: 53 bpm
PR interval: 172 msec
QRS axis: −15°
QRS duration: 105 msec
Sinus rhythm, bradycardia
The QRS duration is mildly prolonged.
The QRS axis is slightly shifted to the left.
R1 + S3 greater than 25 mm and RaVL greater than 11 mm suggest LV hypertrophy.
No LA overload
Comments: The sinus bradycardia reflects atenolol therapy.
CHEST X-RAY
FINDINGS
Cardiothoracic ratio: 51%
The CXR shows a normal heart size. Rib notching is present in the left fifth and seventh ribs. The fullness to the right of the midline may indicate an ascending aortopathy.
Comments: The characteristic “figure 3 sign” delineating the dilatation of the proximal descending aorta and postcoarctation dilatation of the descending aorta creating a double contour in the region of the coarctation is also present.
In most adults with aortic coarctation rib notching is absent or unappreciated. If present, it is often most visible at the posteroinferior border of the third to eighth ribs and is caused by the impression of enlarged and tortuous intercostal collateral arteries.
ECHOCARDIOGRAM
OVERALL FINDINGS
The patient had a bicuspid aortic valve with normal function. There was normal LV size and systolic function, normal function of all other valves, and no chamber enlargement.
FINDINGS
The suprasternal view shows localized segmental narrowing in aortic luminal diameter in the region of the ligamentum arteriosum, just distal to the left subclavian artery.
Comments: The site of the stenosis can only be seen properly in the suprasternal view. However, adult images are not always optimal because of artifact caused by the proximity of the left bronchus.
Even though the coarctation is near the left subclavian, there was still normal blood pressure in the left arm, as may be seen in coarctation, in contrast to the blood pressure in the legs.
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
