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AORTIC COARCTATION – REPORT CASES


Doina Butcovan¹, Gr. Tinica², E. Sandica², V. Diaconescu²,
Cleopatra Borza,  GIM Georgescu²
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The early repair of the coarctation is associated with few perioperative complications and better long term outcome. Therefore, early detection and treatment of coarctation patients is extremely desirable. The aim of the study was to review the effectiveness of neonatal screening examination about this abnormality, the mode and age of presentation, differences in presentation between cases with isolated coarctation, and cases associated with other cardiac lesions.  This was a retrospective study of 6 patients admitted to our hospital, Cardiology Center Iasi, between November 2001 and November 2002, who were found to have coarctation. Referral data were analysed and compared to hospital cardiac evaluation findings. The morphological study was made on surgical biopsic specimens, obtained at surgery, using routine morphological techniques. The microscopical coarctation examination revealed characteristic morphological aspects corresponding to fibro-elastic-muscular proliferation.The importance of the neonatal screening examination of the measuring blood pressure in the limbs to detect coarctation early is needed. Timing of the neonatal screening examination between the third day and the third week is recommended The study revealed the importance of establishing the disease diagnosis for indicating a target effective treatment. Key words: coarctation, femoral pulse, cardiac murmur, screening examination.
 

INTRODUCTION
     Congenital heart diseases (CHD) are the most common congenital malformations, accounting for 9% of all infants deaths and 40% of deaths within this group (1, 2). Left ventricular outflow tract obstruction occurs in 6/10.000 live births, or 7% of patients with CHD (2). Screening for CHD is one of the main reasons for routine examination of babies in the neonatal period and in the earlier infancy (3). It is known that coarctation is a congenital narrowing of the aortic arch lumen as a tube-shaped or a lumenal curtain projecting toward the arterial channel; the significant narrowing has over 50% from arterial circumference (4, 5). Frequently is located distal of left subclavicular artery origin (6, 7). Detection of aortic coarctation (AC) prior to the onset of symptoms should result in earlier referral, lower perioperative morbidity and mortality and better long-term outcome (3). 
     The incidence of the coarctation is about 5-8% of all CHD, being of twice more frequent at men (2). Could be isolated (82%, M/F=2/1) or associated with other CHD (18%, M/F-1/1), from that 11% are represented by ventricular septal defect (VSD) and 7% are other CHD (Persistent ductus arteriosus-PDA, bicuspid aortic valve-BAV, mitral stenosis-MS) (2). From this reason and frequency considerations are recognised 3 main clinical types: 1) isolated coarctation, 2) coarctation associated with VSD, and 3) coarctation associated with complex CHD (1). In AC embryology, there are recognised two main theories: firstly, circulatory theory, which takes in discussion the AC size, referring to the amount of blood reaching in systemic circulation; it is sustained by the presence of the associated lesions (VSD, BAV, AS, MS), and secondly, ductal ring theory; it takes in consideration that normaly, the ductal tissue invades the aortic wall (1, 2). In normal aorta, the ductal tissue affects less than 30% from aortic circumference, while in AC the ductal tissue involvement is over 50% reaching entire aortic circumferencelike an egg-shell (2). 

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University of Medicine and Pharmacy
Faculty of Medicine
¹Department of Pathology
²Cardiology Center Iasi

     Clinical picture has a bimodal appearance: infants are presenting with early clinical manifestations, which are more severe as in adult life, resulting cardiac failure and circulatory collapse, and adults are presenting later in life with a characteristic clinical picture (8, 9). It has been recognized that weak or absent femoral pulse, upper limb hypertension and systolic pressure gradient (SPG) between upper and lower limbs are sensitive screening indicators for coarctation (3). Unfortunately, CHD may escape detection in the neonatal and six week period examination, especially if they are not properly performed (3). The treatment is recommended for avoiding different complications, such as left ventricular insufficiency, cerebral vessels rupture with secondary hemorrhages, renal failure and acidosis (10, 11).
 
 

MATERIALS AND METHODS
    The records of the patients admitted to CCI between November 2001-to November 2002 were reviewed and six patients with diagnosis of left ventricular outflow tract obstruction were found. Data recorded included age, sex, cause of referral, and clinical signs (femural pulse, blood pressure and cardiac murmur). The patients were classified in two groups: the first group was made up of patients with isolated coarctation (3 from 6, 50%) and the second group of patients had coarctation with other cardiac lesions (3 from 6, 50%). Blood pressure (BP) readings were considered correct if measured in the four limbs in the same sittings by oscilometric methods and clearly documented in the file. This was done in all 6 patients. The BP readings were excluded since they were not clearly recorded, including those measured in less than 3 limbs and those measured manually and not using oscilometric methods. Echocardiography and cardiac catheterization were available in our center, as well. The obtained surgical biopsic specimens, were fixed in buffered 10% formalin for 12 hours and quite totally sampled. Samples were processed using routine paraffin embedding, cutting (5 mm) and hematoxilin-eosin (HE) and Van Gieson (VG) staining. 
 

RESULTS AND DISCUSSIONS 
     The study represents 6 case reports of coarctation of adult type, who were presenting with progressive dispnoea having the previous diagnosis of coarctation, established few time ago. The patients, 4 men and 2 women with ages between 9 and 47 years, had an known aortic coarctation history (tabel 1) .
    Ing (3) described 11 patients presented below the age of one month, which reflected the time of onset of symptoms, and thus the severity of their lesions, rather than successful detection in neonatal screening examination, as none of them was asymptomatic at the time of referral. In our study, the patients were presenting for hypertension, breathlessness, abnormal femoral pulse, cardiac murmur and nonspecific symptoms (tabel 2) 
     Clinical examination revealed upper leg hipertension, a sistolic murmur on the left sternum side, cardiomegaly, lower leg hipotension and femoral blood pulse absence. According with Maron (10), the cardinal features of coarctation are varying in sensitivity (tabel 3) 

1. Femural pulse (FP). In Symmers’appreciation (4), palpation of FP is a reliable method of screening for coarctation in children. This was abnormal to all our patients (100%) and 92% in a study by Ing et al (3), reported as absent (7), weak (4), but never normal.

2. Cardiac murmur (CM). Although 100% of patients in the Ing study (3) had a murmur, only 66,66% (4 cases) of our patients had murmur. 

3. Systolic hypertention (SH). SH was found in 45 from 51 patients (94%) in the Ing study (3), but only in 5 from 6 patients (66,66%) in our study. 
     A comparison between clinical findings in our study and those of Ing (3) is presented in table 3. He considers that the most reliable clinical sign for detection of coarctation is systolic blood pressure gradient between the arms and legs. Ing appreciated, blood pressure measurement is not difficult with  the availability of automated oscilometric blood pressure machines, being highly accurate in recording both upper and lower extremity blood pressure in the new born infant patients with coarctation associated with other cardiac lesions than patients with isolated coarctation. He concluded, SPG in the lower range of significance can be considered of indicator for complex cardiac lesions rather than isolated coarctation in symptomatic patients below the age of one year. In our study the diagnosis was made on echocardiography and X ray chest results, confirming left ventricular hypertrophy, echocardiography evitentiating the AC of different ages, cardiac catheterization for revealing the AC and an permanent increased pressure gradient at the level of AC. X ray chest evidentiated costal erosions in 3 cases, as well.
     The present study describes cases of adult type of aortic coarctation, which were diagnosed at the onset of the clinical manifestations, representing the proper timing for the sugery for preventing the complications. So, many studies (3, 11) have indicated that delayed repair of coarctation is associated with an increased morbidity and mortality because of cardiovascular diseases in adult life. Furthmore, it has been stated that repair during early childhood with less systemic hypertention and improved long-term outcome (10). In our study, the surgery was indicated at all diagnosed hipertension cases due to the risc of complications, while at new borns, from Ing study (3), the surgery was indicated immediately, in cases with severe cardiac insufficiency, or at 3-6 months in the absence of the cardiac insufficiency. 
     Generally, the operation was the coarctation resection with end to end anastomosis or resection and vascular graft interposition. Sometimes, the coarcatation reccurence could follow at 3 months post-operation (1), due to an incomplete resection of the coarctation tissue; in this case it could be corrected by baloon angioplasty. Morphologically, we demonstrated a diaphragm coarctation, centred by an oval or triungular hoal (fig.1)  and a thickened muscular wall (fig. 2) , occupying over 75% from wall circumferenceand having an intimal fibro-muscular structure (fig. 3, 4) .
 

CONCLUSIONS
     The importance of the neonatal screening examination and of measuring BP in all limbs to detect early coarctation is vital. Timing the neonatal screening examination between the third day and third week after delivery is recommended. Any child with hypertension should specifically evaluated for coarctation. SPG between upper and lower limbs is a sensitive indicator for coarctation and it is recommended to be used as a screening test. 
 

REFFERENCES
1. Silver MD. Aortic coarctation. In Silver MD. Gotlieb AI. Eds. Cardiovascular Pathology. Churchill Livingstone 2001, 385-389. 
2. Virmani R. Coarctation of the aorta. In: Allen Burke and Renu Virmani. Atlas of Cardiovascular Pathology. Ed. AFIP. Washington DC 1966: 469-471.
3. Cohen M, Foster V, Steel PM. Coarctation of the aorta. Long-term follow up and prediction of outcome after surgical correction. Circulation 1989; 80: 840-5
4. Symmers WSC.  The coarctation of the aorta. In: Symmers WSC. Cardiovascular System. Springer-Verlag, New York, 1980, 672-675. 
5. Waller BF.CHD. In: Waller BF. Pathology of the Heart and great vessels, The Mosby Company, 1998, 1173-1178.
6. Olsen EGJ. Coarctation of the aorta. In: Olsen EGJ. Pathology of the Heart, The Macmillian Press LTD, 1980, 282-293
7. Colin MB. Aortic coarctation. In: Colin MB Ed. Heart Pathology.  Churchill Livingstone  1980, 2xx-2yy.
8. Gross RE. Coarctation of the aorta. In: Sternberg SS. Diagnostic Surgical Pathology. Ed. Raven Press, 1944: 1196-1121.
9. Reid JV. Aortic coarctation. In: Silverberg SG. Principles and Practice of Surgical Pathology and Cytopathology. Ed. Churchill Livingstone 1997, 113-132.
10. Maron BY, Humphries YO, Rowe RD. Prognosis of surgically corrected coarctation of the aorta: 120 year postoperative appraisal. Circulation 1973; 47: 119-26
11. Stein RL. CHD study group. Resources for optimal long-term care of CHD. Circulation 1971; 44: 1205
 
 

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 e-mail: butcovan@yahoo.com

 
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