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Juxtaductal aortic coarctation. Analysis of 84 patients studied hemodynamically, angiographically, and
Insights
This study correlates clinical, hemodynamic, and anatomic factors in juxtaductal coarctation of the aorta. Findings reveal key anatomic causes and surgical outcomes, improving understanding of aortic coarctation management.
Area of Science:
- Cardiology
- Vascular Surgery
- Pediatric Cardiology
Background:
- Coarctation of the aorta is a congenital heart defect with varied clinical presentations.
- Previous studies have not comprehensively correlated clinical, hemodynamic, angiographic, anatomic, and operative findings in juxtaductal coarctation.
- Understanding these correlations is crucial for effective diagnosis and treatment.
Purpose of the Study:
- To correlate clinical, hemodynamic, angiographic, anatomic, and operative findings in patients with juxtaductal coarctation of the aorta.
- To identify the primary anatomic factors contributing to aortic coarctation.
- To evaluate early and late postoperative outcomes following surgical repair.
Main Methods:
- Retrospective analysis of 84 patients (age 1-49 years) with juxtaductal coarctation.
- Clinical data collection including blood pressure, physical examination (femoral pulsations), and murmurs.
- Hemodynamic assessment via pressure gradients, radiographic imaging (rib notching, cardiomegaly), electrocardiography, and angiographic measurements.
- Anatomic evaluation of 70 excised coarctations via serial sectioning.
- Correlation of these findings with operative repair and postoperative outcomes.
Main Results:
- All patients presented with murmurs; 76% had diminished or absent femoral pulsations. 50 patients had elevated arm systolic blood pressure (>140 mm Hg).
- Average peak systolic pressure gradient across the coarctation was 45 mm Hg. Rib notching correlated with age and coarctation diameter.
- The primary anatomic cause was medial invagination, with intimal proliferation contributing to narrowing. Early mortality was 4.3% (3/70), primarily in patients with associated anomalies. Postoperative blood pressure improved in most survivors, though 25% had late elevated systolic pressure.
Conclusions:
- Juxtaductal coarctation of the aorta presents with characteristic clinical and hemodynamic findings.
- Medial invagination is the principal anatomic factor, often exacerbated by intimal proliferation.
- Surgical repair leads to significant blood pressure reduction, but long-term monitoring for hypertension is necessary.
Abstract:
Although many studies of juxtaductal coarctation of the aorta have been reported, none has correlated clinical, hemodynamic, angiographic, anatomic, and operative findings. Of 84 patients (62 male and 22 female; age range, 1 to 49 years [mean 17]), all had murmurs; 76 had absent, diminished, or delayed femoral pulsations; 50 had cuff systolic blood pressures in the arm greater than 140 mm Hg, and 30 had diastolic pressures greater than 90 mm Hg. The average pressure gradients (mm Hg) by direct measurements above and below the coarctation in 35 patients were peak systolic, 45; mean, 17; and diastolic, 5. Rib notching, visible in chest roentgenograms in 43 patients, correlated directly with age and inversely with the diameter of the coarctation. Moderate or marked cardiomegaly by radiograph was present in only 1 of 48 patients with isolated coarctation and in 17 of 36 with associated cardiovascular malformations. Electrocardiograms were abnormal in more than two thirds of patients with associated anomalies, but were normal in more than three fourths of those with isolated coarctation. In 70 excised, serially sectioned coarctations the aortic lumens were completely occluded in 4 patients, up to 0.5 mm in internal diameter in 22 patients, from 0.6 to 2 mm in 26 patients, from 2.1 to 5 mm in 14, and greater than 5 mm in 4, and correlated directly with lumens measured angiographically. The most significant anatomic factor causing the coarctation was invagination of the media from the posterior aortic wall, but intimal proliferation (jet lesion) at and immediately distal to the invagination contributed to the narrowing. Three (each with associated anomalies) of 70 patients died early after coarctation repair. Systolic or diastolic blood pressures decreased early postoperatively in 58 (87%) of 67 surviving patients, and both pressures decreased in 42 (63%). Late postoperatively (mean follow-up, 4.7 years), the systolic blood pressure remained elevated in 25% of patients.
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