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AORTIC COARCTATION – REPORT
CASES
Doina Butcovan¹, Gr. Tinica²,
E. Sandica², V. Diaconescu²,
Cleopatra Borza, GIM Georgescu²
...
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).
- - -
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.
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