The unusual hypertensive patient

Abstract

Many young patients with systemic hypertension have secondary causes which if identified early could lessen the cardiovascular complications associated with long standing hypertension. Coarctation of the aorta is one of those rare causes. We describe a 40 year old male with hypertension, coarctation of the aorta, bicuspid aortic valve, PDA and an adrenal mass.

Essential systemic hypertension can affect young adults, but many have secondary causes most frequently due to chronic renal or renovascular disease.

Coarctation of the aorta is a rare cause of systemic hypertension. Most people with untreated aortic coarctation die young before the age of 50 years, due to severe cardiovascular complications. Though the association of adrenal mass and or pheochromocytoma have been previously reported in literature [1], this to our knowledge is the first in our community.

We describe a male patient 40 years of age where we diagnosed congenital cardiovascular disease and a rare association. We also reviewed the literature on this anomaly and its associations.

Case report

A 40 year old man was referred to the cardiology clinic at KAMC-Jeddah for further treatment and evaluation of long standing hypertension which was diagnosed at the young age of 32. The patient was complaining of occasional headaches. He denied episodic palpitations, diaphoresis, facial flushing and weight loss. He also denied history of intermittent claudication. His treatment consisted of amlodipine 10 mg, indapamide 1.5 mg and lisiopril 10 mg daily.

On physical examination, he was well looking, there was no dysmorphic features. Blood pressure in the right arm was 153/78 with no difference from the left upper extremity.

There was more than 40 mmHg systolic gradient between the upper and the lower extremities.

His radial pulse was strong at a rate of 78 b/m with bilateral weak femoral pulses and significant radio-femoral delay. The cardiac auscultation revealed normal first heart sound loud second heart sound ejection click and diamond shaped systolic murmur 2/6 best heard in the L parasternal and interscapular region.

The electrocardiogram showed sinus rhythm with LVH by voltage criteria. Renal function and electrolytes were normal. Chest X-ray showed normal cardiac silhouette, rib erosions were not appreciated. Echocardiagram revealed a normal left ventricular function, concentric LVH, bicuspid aortic valve with mild stenosis (AVA OF 1.8 cm² and mean gradient of 22 mmHg).

Suprasternal window confirmed the presence of coarctation with ∼57 mmHg pressure gradient across it (Fig. 1). (Hemodynamically significant) no other anomalies noted.

Figure 1.

Suprasternal notch window showing flow turbulence in the descending aorta and flow acceleration with a gradient of 57–54 mmHg (significant more than 20 mmHg).

Aortic CT angiogram not only confirmed the diagnoses (Fig. 2) but also showed an incidental finding of right adrenal mass of 3.7 × 5.2 cm confirmed by CT abdomen (Fig. 3) suggesting possible coexistence of phoechromocytoma.

Figure 2.

Volume rendering 3 D reconstruction showing the site of the coarctation.

Figure 3.

CT abdomen showing large right adrenal mass with calcification.

Full endocrionological assays excluded Cushing and pheochromocytoma.

The patient treatment was modified indapamide was discontinued labetalol 200 mg twice daily was added together with Lipitor 40 mg.

The patient underwent laparoscopic right adrenalectomy due to a large sized mass. Histopathology of the mass showed cystic changes with calcification there was no malignancy.

Cath lap angiographic study showed atretic PDA at the site of the coarctation (Fig. 4). He underwent successful covered stent placement at KAMC-Riyadh that closed the PDA and opened up the coarctation segment. Follow up aortic CT angiogram confirmed patency of the stent, shrinkage of the size of the collaterals and absence of stent related complications (Fig. 5).

Figure 4.

LAO aortic root angiogram showing the coarctation and the PDA (a).

Figure 5.

Showing the stent at the site of the coarctation (under expanded) there was no significant gradient following deployment.

We were able to decrease his antihypertensive medication to atenolol 50 mg daily and lisiopril 10 mg daily in addition to Lipitor and aspirin.

He however will be followed up regularly with yearly echocardiogram to monitor progression of his bicuspid aortic valve stenosis as well as assessment of the need of further stent dilatation. He likely would require aortic valve surgery in the future.

Discussion

Native coarctation of the aorta has the prevalence of 5–8% [2] this involves stenosis of the distal part of the aortic arch after the bifurcation of the left subclavian artery at the level of the ductus arteriosus, resulting in raised blood pressure at the upper limbs.

Isolated coarctation is slightly more common in males than females, especially in older patients. It is frequently associated with other congenital defects and as many as 50–80% will have a bicuspid aortic valve [3]. Other cardiac defects are abnormalities of the mitral valve (prolapsed and stenosis), patent ductus arteriosus, a ventricular septal defect, endocardialendoelastosis. Non cardiac association includes the presence of cerebral aneurysms in the circle of willis. Hypospadias, club foot, ocular defect and pheochromocytoma. Coarctation is the most common congenital defect in patients who have XO turner’s syndrome.

Coarctation of the aorta classically produces symptoms at two stages of life, in early infancy and in the second and third decades [4]. Symptoms develop in infancy in only approximately 10% of all patients with an uncomplicated coarctation and they are related to proximal aortic hypertension and under perfusion of the distal aorta. Symptoms usually remain minimal until the second or third decade of life, when the ravages of prolonged hypertension become manifest and associated complications emerge. Rupture of the aorta or dissecting aneurysm is most commonly seen in the third and fourth decades of life [5]. Infective endocarditis also becomes a more serious complication with age [6], often affecting the associated bicuspid aortic valve. Similarly, rupture of an aneurysm of the circle of Willis is more evident later in life and is not always related to the presence of hypertension [7]. Premature coronary artery disease may also occur [8]. Ideally aortic coarctation should be detected during early infancy by routine physical examination of upper and lower-extremity pulses. Although most people with systemic hypertension have essential hypertension, aortic coarctation still remains a very rare (0.2% of hypertensive subjects [9]) but potentially reversible cause of hypertension if diagnosed and treated in early life.

Prognosis of an unrepaired coarctation is very unfavorable. Approximately 25% die between 20 and 50 years of age, while 90% of the original group dies before 50 years of age. Another 5% die at 50–60 years and the remaining 5% die at still older age [6,4].

In children and adults, coarctation should be corrected electively when the diagnosis is made, regardless of acquired complications. Most authors believe that children with uncomplicated asymptomatic coarctation are best operated between age 3 and 5 years, when operative risk is low and the incidence of postoperative complications is acceptable [8]. An increased preoperative mortality in corrections performed in patients older than age 40 years has been reported [10]. The incidence of residual postoperative complications, i.e., persistent hypertension, paraplegia, and mesenteric arthritis, is higher in these older patients [10]. However, in the absence of precluding medical problems, age alone is not a contraindication to repair.

Correction after the age of 5 years results in a considerable risk for persistent hypertension, especially exercise-induced hypertension [11]. When operation is performed under 2 years of age there is an increased risk for recoarctation [12]. Surgical repair has been the standard therapy for coarctation since its introduction in 1945 [13]. A variety of surgical approaches can be used, including resection and end-to-end anastomosis, the use of the left subclavian artery as a patch, or a Dacron patch. Although there are some reports of surgical treatment with good outcome [14,15], even in combined surgery with CABG [16], it still remains a very high risk intervention that must be weighed against the benefit of prolonging a patient’s survival and ameliorating a patient’s clinical symptoms.

Balloon angioplasty for the correction of aortic coarctation was first introduced in 1982. Many now recommend this technique as an alternative for surgery or recoarctation of previously operated coarctation [17,18]. Some extend this preference to the unoperated or native coarctation [19–22].

Nowadays Stent implantation is considered the therapy of first choice for aortic coarctation in adults [23–25]. The advantages of this method have extended its use to adolescents and children [26] and in some centers even to small infants.

Conclusion

Young patient have essential hypertension, but many have secondary causes, every effort has to be exhausted to excluded secondary causes. This case highlights the importance of thorough physical examination as it did reveal the diagnosis. We think the adrenal mass presence is just an incidental finding.