Secondary hypertension and aortic coarctation in a young adult: A case report

Abstract

Aortic coarctation is a rare cause of secondary hypertension (<1% cases) and can be challenging to detect due to its few clinical manifestations. Early diagnosis and treatment are important because patients with unmanaged aortic coarctation are at increased risk of cardiovascular complications and have a reduced life expectancy. We describe a case of secondary hypertension in a young adult female caused by aortic coarctation, first detected in a general practitioner setting, resulting in the need for a left subclavian-carotid bypass vascular surgery and a descending aortic stent vascular surgery. This case highlights the critical role that proximity medicine in general practice can have in improving the early detection of clinically silent conditions by routinely monitoring blood pressure and other vital parameters, and the increasing importance of medical imaging in assisting early diagnosis and guiding the surgical management of complex cases.

Introduction

Most of Portugal's hypertensive population is managed at the primary care level [1]. Arterial hypertension is one of the most important cardiovascular risk factors for events such as stroke and myocardial infarction, with 5%-10% of cases being caused by secondary hypertension. Early detection and treatment are particularly vital in this form of hypertension, as it mostly comprises underlying treatable causes [1], [2], [3].

The aim of this report is to describe a case of secondary hypertension caused by aortic coartation (AoC) in a young adult female, which was detected in a primary care setting. We followed the CARE case report guidelines when producing this report.

Case report

March 2022—patient information, clinical findings, and diagnostic assessment

In March 2022, a 23-year-old female attended with her general practitioner reporting previous episodes of palpitations with thoracic pain, with no irradiation or relation to physical exertion, and which resolved spontaneously within minutes. The clinical presentation progressed to a strong pulsating headache. There was no record of significant personal or family medical history, with the patient only reporting taking a birth control pill (0.02 mg of ethinylestradiol with 0.075 mg of gestodene). During physical examination, a 3 to 5 grade systolic heart murmur was auscultated, associated with a blood pressure of 200/104 mmHg and a heart rate of 79 BPM, in the left brachial artery. After asking the patient to rest for a few minutes in a quieter area, we took another blood pressure measurement, which measured 182/97 mmHg, and a heart rate of 81 BPM. Blood pressure in the lower limbs was around 135-145/85-95 mmHg with a decreased bilateral femoral pulse. The patient was referred to the nearest hospital emergency service, with a follow-up appointment also being made at the GP practice.

At the GP follow up appointment, the patient reported that her clinical hypertensive presentation was dismissed at the emergency services, and that she had received a diagnosis of anxiety and a prescription for a short-acting benzodiazepine. During the GP physical examination her blood pressure was 182/109 mmHg with a heart rate of 75 BPM in the left brachial artery and a 3 to 5 grade systolic heart murmur. We ordered several exams (see Table 1) to screen for causes of secondary hypertension and prescribed Olmesartan 10 mg once a day. The patient returned one month later, reporting home blood pressure measurements in the range of 135-145/85-95 mmHg after starting the anti-hypertensive medication. Neither the electrocardiogram nor the chest radiography showed abnormal results. The echocardiogram report described a bicuspid aortic valve and a suprasternal gradient of 23 mmHg at the beginning of the descending aorta, and the renal Eco-Doppler report described bilateral diminished resistance fluxes, both of which are suggestive of aortic coarctation. Given this information and considering the limitations of the local resources of the national health service, we requested an appointment in a vascular surgery unit in Lisbon.

Table 1.

Resume of the requested exams during this case.

Requested exams during this case

-Bloodwork analysis: hemogram, creatinine, total cholesterol, HDL cholesterol, triglycerides, LDL cholesterol, gamma-glutamyl transferase, aspartate aminotransferase, aspartate transaminase, thyroid stimulating hormone (TSH), thyroxine (T4) and catecholamines -Electrocardiogram -Echocardiogram -Chest Radiography -Eco-Doppler of renal arteries -Thoracic computer tomography -Thoracic magnetic resonance -Thoracic angio-magnetic resonance imaging (Angio-MRI) with Maximum Intensity Projection (MIP) -Post-surgery Vascular Doppler Ultrasound of the upper limbs

In June 2022, the patient had another follow-up consultation with her GP to prepare for the vascular surgery scheduled in the hospital setting in Lisbon, and to show her the results of the thoracic Angio-Magnetic Resonance Imaging (Angio-MRI) exam. This exam (see Fig. 1) showed a significant stenosis of the descending aorta, right after the left subclavian artery origin, with an almost laminar aortic flow in the narrowed artery, translating a significant aortic coarctation. These results corroborated the initial findings of the echocardiography and the renal Eco-Doppler.

Fig. 1.

(A) Coronal reconstruction of a thoracic angio-magnetic resonance image (Angio-MRI) showing a clear hourglass shape caused by a significant stenosis of the descending aorta right after the left subclavian artery origin, compatible with a significant aortic coarctation. (B) The same thoracic angio-magnetic resonance image (Angio-MRI), but with Maximum Intensity Projection (MIP)—coronal reconstruction.

January 2023—therapeutic intervention

The patient had a left subclavian-carotid bypass vascular surgery along with a descending aortic stent in early January 2023. Fig. 2 shows the comparison of the descending aorta before and after the vascular surgery. Fig. 3 shows the post-surgical vascular Doppler and its peak flow velocity in the descending aorta and left subclavian artery. This exam displayed monophasic arterial curves in the left subclavian artery, and biphasic arterial curves at the level of the left axillar and humeral artery, indicating reduced arterial amplitude.

Fig. 2.

(A) Magnetic resonance data for sagittal reconstruction, showing a narrowing of the descending aorta right after the left subclavian artery origin, compatible with a significant aortic coarctation. (B) Computed tomography data of chest for sagittal reconstruction, after the left subclavian-carotid bypass vascular surgery, showing the descending aortic stent.

Fig. 3.

Doppler ultrasound in spectral mode, for assessment of descending aortic (A) and left subclavian (B) peak flow velocity, after the left subclavian-carotid bypass vascular surgery and the placement of descending aortic stent.

Discussion

Aortic coarctation (AoC) accounts for less than 1% of cases of secondary hypertension [4] but 4%-6% of cases of congenital heart defects; as such, it is one of the most common congenital cardiac anomalies. It causes a narrowing of the descending aorta, usually located at the insertion of the ductus arteriosus distal to the left subclavian artery, which typically results in a left ventricular pressure overload [5,6]. It is often diagnosed during childhood, but some patients may remain apparently asymptomatic until adulthood due to its few clinical manifestations, which may not be detected during a physical exam only [4,7,8]. Undetected severe hypertension caused by AoC may lead to headache, epistaxis, or heart failure. The reduced blood flow may also induce intermittent claudication, particularly during physical exertion [5].

The prognosis for patients with untreated coarctation of the aorta is dismal, with a 75 percent mortality rate by 46 years of age [5,9]. Successful early surgical repair is associated with a 10-year survival rate of over 90% [6]. Therefore, general practitioners must be aware of the signs and symptoms of secondary hypertension, and should monitor blood pressure opportunistically [1].

Medical imaging was pivotal in achieving the correct diagnosis for this patient (as shown in Fig. 1, Fig. 2), thus emphasizing its critical role in current medicine. Angio-CT and angio-MRI are the diagnostic gold standards for this condition [6], but are only available in hospital settings in the Portuguese National Health Service. This shortage of resources poses additional barriers to a timely diagnosis.

The Angio-MRI was chosen due to its hemodynamic assessment capabilities [10], allowing the determination of aortic stiffness [11], which is a key feature for establishing the best treatment plan in this clinical situation and monitoring for the possible adverse hemodynamic consequences of a residual gradient or inadequately controlled hypertension [12]. The current evidence does not indicate significant differences between stent, balloon, or surgical treatment modalities in terms of prognostic outcomes, leaving a choice of therapeutic approach open to the vascular team [12]. In the future, the use of advanced imaging techniques like the 4D flow MRI, combining noninvasive anatomical and hemodynamic data, will improve prognosis for these patients [11,12], aiding the vascular surgery team's decision-making process regarding the best approach for optimal outcomes.

This clinical case highlights several main points. First, it demonstrates the critical role that proximity medicine in general practice can have in improving the early detection of clinically silent conditions by routinely monitoring patients’ blood pressure and other vital parameters. Second, it shows the importance of medical imaging in assisting early diagnosis and in guiding the surgical management of complex cases.

Ethical approval

Not applicable.

Patient consent

The author declares to have informed written consent of the patient, for the submission and publication of the article.