Chatzistamatioy EI1*, Moustakas GN2, Avgeropoulou C1, Androulakis A1, Tousoulis D3 and Kallikazaros IE1
1Cardiology Department, Hippocration Hospital, Athens, Greece
2Cardiology Clinic, Sismanoglio Hospital, Athens, Greece
3First Cardiology Clinic, University of Athens, Hippocration Hospital, Athens, Greece
Received:20 July, 2015; Accepted: 12 October, 2015; Published: 16 October, 2015
Evangelos I Chatzistamatiou, Kaisareias street, Nr. 6, Ampelokipoi, P.B: 11527, Athens Greece, Tel: +306937433637; E-mail:
Chatzistamatioy EI, Moustakas GN, Avgeropoulou C, Androulakis A, Tousoulis D, et al. (2015) Middle Aortic Syndrome: A Case Report and Review of the Literature. Global J Med Clin Case Reports 2(1): 013-016. DOI: 10.17352/2455-5282.000019
© 2015 Chatzistamatioy EI. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Coarctation of the aorta is a relatively common defect that accounts for 5-8% of all congenital heart defects and is characterized by discrete medial thickening with superimposed neointimal tissue, leading to aortic lumen narrowing of different degrees.
Today's knowledge is that the majority of lesions are juxtaductal, with the classic coarctation located in the thoracic aorta distal to the origin of the left subclavian artery at about the level of the ductal structure . However, a coarcted segment may be present in the distal descending thoracic or abdominal aorta and is referred as Middle Aortic Syndrome (MAS). This entity is extremely rare, representing only 0.5-2% of all aortic coarctation cases , with total number of published patients not exceeding three hundred. Congenital, acquired, inflammatory, and infectious etiologies have been proposed.
Regardless of the location, long lasting coarctation increases significantly wall stress of the left ventricle and arterial tree proximal to stenosis, leading to ventricular hypertrophy, heart failure, reactive vascular hypertrophy and premature atherosclerosis. Moreover, hypoperfusion distal to stenosis may lead to renal, visceral and lower limb ischemia .
We present a case of a middle aged woman with asymptomatic congenital thoraco-abdominal aortic dysplasia, newly discovered mild hypertension and premature carotid atherosclerosis.
A 55-year old white female, civil servant, postmenopausal the last 5 years, without hormone replacement therapy was admitted to our antihypertensive unit with a three month history of newly discovered, stage 1, systemic hypertension. In the coming weeks systematically monitored the values of BP and found that morning measurements ranged to 140/75 mmHg and evening measurements to 145-150/75 mmHg. She has occasionally complained of headache and dizziness.
The patient had a positive familial history for hypertension by both parents. She reported normal birth weight, but her's mother age at the time of delivery was only 13 years old. Her personal medical history was negative for sleep disturbances or intake of any substances and medications that increase BP levels. Moreover, she was never smoker, with reported three abortions at a young age, low salt consumption and physically active (intense aerobic exercise at a regular basis).
The physical examination demonstrated that the patient was in good condition with normal body adiposity (BMI 22.5 kg/m2, waist circumference 76 cm). Both the office systolic and diastolic blood pressures and heart rate were in the normal range in both arms (mean 125/75 mmHg, 65 bpm). Complete clinical evaluation was negative for signs and symptoms suggestive of secondary forms of hypertension, except slightly diminished and delayed femoral pulses.
Twelve-lead resting ECG revealed sinus rhythm at 70 bpm, normal electrical axis at 75o, without signs of atrial enlargement, LV hypertrophy or ST-T wave abnormalities (Sokolow-Lyon index: 2.2 mV). Blood and 24hour urine collection tests were negative for endocrine hypertension. Fasting plasma glucose was 96 mg/dl, LDL-Cholesterol: 120 mg/dl, HDL-Cholesterol: 67 mg/dl, triglycerides: 76 mg/dl, eGFR: 91.5 ml/min/ 1.73m2 and Albumin to Creatinine ratio: 4.41 mg/gr.
Despite that 24hour ambulatory blood pressure monitoring presented normal BP values during the day (128/69 mmHg) and night (104/56 mmHg), serial home BP measurements during several weeks were abnormal (morning 140/75 mmHg, evening 150/80 mmHg), setting the diagnosis of masked essential hypertension stage 1.
Fundoscopic examination revealed beading of the middle and distal portion of the retinal veins, without clear evidence of hypertensive signs (Figure 1). Symptom-limited maximal treadmill exercise test with Bruce protocol was negative for ischemia. The duration of exercise was 8΄30΄΄ (10 METS), BP and heart rate rose to 200/90 mmHg and 164 bpm, respectively. Conventional and Tissue Doppler echocardiographic study was normal: Ascending thoracic aorta was 3.2 cm, Left atrial volume index was 19 cm3/m2, and Left ventricular mass index was 60.7 gr/m2, Ejection fraction=65%, Transmitral E/A=1.42, TDI basal lateral E΄=11 cm/sec and A΄=7 cm/sec.