Coarctation of Aorta Presenting as Spontaneous Subarachnoid Hemorrhage in a Young Male: A Case Report

Emergency and Critical Care Team, Kauvery Hospital, Cantonment, Trichy, India

Abstract

Coarctation of the aorta (CoA) is one of the common congenital cardiovascular diseases. CoA can be diagnosed over a wide range of ages, with varying degrees of severity, and in various presentations. The most common presenting age of CoA is 3-6months with only a small proportion of adolescent and adults diagnosed with primary coarctation of the aorta. Intracranial aneurysm that undergo spontaneous rupture causing subarachnoid hemorrhage (SAH) are rare in adult patients with CoA. We report a 41-year- young male with CoA and SAH who successfully underwent right pterional craniotomy and clipping of an aneurysm.

Background

Coarctation of aorta complicated by subarachnoid hemorrhage is not commonly seen in clinical practice. Subarachnoid hemorrhage accounts for 1-7% of all intracranial hemorrhage. The most common cause of subarachnoid hemorrhage is a head injury, etiology of non-traumatic subarachnoid hemorrhage being rupture of a cerebral vascular aneurysm.

However, an estimated 15-20% of all subarachnoid hemorrhage occurs in the absence of intracranial vascular anomalies. Causes of non-traumatic subarachnoid hemorrhage are likely to be multifactorial; and hypertension corresponds with increased risk. Often fatal, the morbidity associated with subarachnoid hemorrhage correlates with the age at onset and extent of the neurological compromise. CoA which accounts for 5-8% of congenital heart disease, are rarely associated with intracranial hemorrhage and is usually missed in clinical examination. Here we report a case of a young male who presented with long standing uncontrolled hypertension and subarachnoid hemorrhage, who was incidentally diagnosed to have coarctation of aorta when worked up

Case Presentation

A 41-year-aged young male presented at emergency department with severe headache on 22nd April 2022. He was recently diagnosed to have hypertension for last 6 months, but was not on medication.

He had been treated at a local hospital and evaluated where CT revealed subarachnoid hemorrhage with bilateral chronic lacunar infarct.

On clinical evaluation, he had a BP, upper limb, of 150/100mmHg and lower limb 100/70mmHg. Lower limb pulses were feeble bilaterally. Routine blood investigations were within normal limits.

CT brain angiogram revealed saccular Anterior Communicating Artery (ACA) aneurysm with diffuse subarachnoid hemorrhage and mild obstructive hydrocephalus. Patient underwent successful right pterional craniotomy and clipping of the aneurysm.

CoA-1

Fig. 1. ACOM aneurysm.

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Fig. 2. S/P clipping of aneurysm.

On cardiac evaluation- ECG revealed normal sinus rhythm and Left Ventricular Hypertrophy (LVH).

2D echocardiogram showed evidence of coarctation of aorta (gradient 38 mmHg), concentric LVH, good LV function, intact septum, bicuspid aortic valve with an eccentric jet mild aortic regurgitation (AR), dissection flap in the aortic root and decreased flow pattern in descending aorta (abdominal aorta).

CoA-3

CT chest revealed mild cardiomegaly; left ventricle appeared enlarged, no evidence of aortic dilatation.

CoA-4

Patient was subjected for computed tomography (CT)aortography for localizing the site and extent of coarctation of aorta. The CT also showed cardiomegaly, left ventricle enlargement, dilated pulmonary artery, no obvious evidence of dissection, evidence of diffuse thickening with significant luminal narrowing involving the proximal thoracic aorta distal to subclavian origin, post stenotic dilatation, and evidence of inferior rib notching involving the 4-8 ribs on both sides. Diffuse narrowing of bilateral common iliac artery was present with normal opacification of the external iliac artery and common femoral artery.

Patient’s BP was regulated to target. Patient was electively planned for coarctoplasty after 4 weeks.

Discussion

Coarctation of the aorta is characterized by short-segment narrowing in the region of the ligamentum arteriosum adjacent to the origin of the left subclavian artery. Sometimes the constriction occurs proximal to the subclavian artery when it is called pre-ductal coarctation of aorta. The incidence of coarctation of the aorta is 4 in 10,000 live births. The adult who had surgical repair of coarctation of the aorta as an infant is more likely to have associated cardiac lesions like bicuspid aortic valve, subaortic stenosis, ventricular septal defect, and arch hypoplasia of varying degrees. Five to eight percent of children with congenital heart disease have coarctation of aorta.

The defect imposes significant afterload on the left ventricle resulting in wall stress, compensatory left ventricular hypertrophy, left ventricular dysfunction and collateral formation. Coarctation of aorta in adults usually presents as systemic hypertension and discrepancy between upper limb and lower limb blood pressure as in our case.

The coarctation of the aorta may be demonstrated on a suprasternal notch view of the aortic arch and proximal descending aorta, which, when combined with color flow imaging and continuous-wave spectral Doppler interrogation, may demonstrate turbulence in the proximal descending aorta and show the characteristic flow profile of forwarding diastolic flow. An abnormal Doppler flow pattern may also be noted in the abdominal aorta, i.e. decreased pulsatility and absence of early diastolic flow reversal. Abnormal flow in collateral vessels can be detected by color flow and pulse Doppler.

The natural history of unrepaired coarctation of the aorta includes the development of systemic hypertension and subsequent morbidity and death from cardiovascular disease. The age at correction is the most important factor for the relief of hypertension and long-term survival. The incidence of spontaneous subarachnoid hemorrhage is reported to be around 10.5 per 100,000 person-years; and the leading cause of spontaneous subarachnoid hemorrhage is the rupture of an intracranial aneurysm, which accounts for about 80% of cases and has a high rate of death and complications.

In patients with coarctation of the aorta, intracerebral or subarachnoid hemorrhage is more common in the second and third decades. The risk of serious neurological complications associated with coarctation of the aorta and the resultant proximal hypertension mandates early diagnosis and treatment. Berry aneurysms are reported to occur in 10% of patients with coarctation of aorta and this has been related both to the high pressure of the vascular tree proximal to the coarctation of the aorta and to congenital defects of the vascular tree. The 2008 American Heart Association/American College of Cardiology guidelines for intervention in coarctation of aorta includes peak to peak coarctation of aorta gradient ≥ 20 mmHg or peak to peak coarctation of aorta gradient < 20 mm Hg in presence of anatomic imaging revealing significant coarctation of the aorta. The European Society of Cardiology recommends early treatment in all patients with a non-invasive pressure difference of more than 20 mmHg in upper and lower limbs regardless of symptoms but with upper limb hypertension > 140/90 mmHg and significant left ventricular hypertrophy. Balloon angioplasty was introduced in 1982 and is currently done with or without stent deployment. It is the preferred treatment modality in native coarctation of the aorta in adults or re-coarctation of the aorta after surgery. There is an increased incidence of an aneurysm and restenosis after balloon angioplasty. Despite successful repair of coarctation of the aorta, recurrent hypertension is common during long-term follow-up.

Conclusion

The risk of cerebrovascular complications associated with coarctation of the aorta and resultant hypertension mandates early diagnosis and treatment. Intracranial aneurysm rupture associated with coarctation of the aorta has a high early mortality in untreated cases. A ruptured intracranial aneurysm can be successfully treated with surgery or clipping of an aneurysm. In our case, patient successfully underwent right pterional craniotomy, clipping of an aneurysm and electively planned for coarctoplasty after 4 weeks.

References

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