Resistance to captopril in hypertension of coarctation of the aorta

Two patients with hypertension due to coarctation of the aorta were treated for 12 weeks with captopril at increasing doses. After an immediate response, resistance developed to captopril, which was associated with restoration of initially elevated renin and plasma aldosterone levels. Lack of hypertension control may be related to long-term reappearance of mechanisms which lead to excessive renin stimulation.     

Baroreceptor reflex in a patient with coarctation of the aorta

We report the case of a 23-year-old man with coarctation of the aorta whose baroreceptor sensitivity was extremely reduced. Antihypertensive therapy was effectively performed after coarctectomy. Only one month after coarctectomy, baroreflex sensitivity had recovered. The present case is believed to be the first in which reduced baroreceptor sensitivity in coarctation of the aorta was reversible in the early phase after coarctectomy.     

Echocardiographic indices of Doppler flow patterns compared with MRI or angiographic measurements to detect significant coarctation of the aorta

Evaluation for the presence and severity of coarctation of the aorta (CoA) by two-dimensional echocardiography alone can be difficult. The purpose of this study was to use Doppler velocity and pressure gradient half-time in systole and diastole to estimate CoA severity. Doppler echocardiograms of children with suspected CoA and either an aortic angiogram or thoracic magnetic resonance imaging (MRI) performed within 1 month of the echocardiogram were evaluated. Patients with patent ductus arteriosus, significant aortic insufficiency, long tubular CoA, or CoA outside the thorax were excluded. Measured Doppler variables, indexed for heart rate, included systolic velocity half-time (sVHTi), diastolic velocity half-time (dVHTi), systolic pressure half-time (sPHTi), and diastolic pressure half-time (dPHTi). For each of these variables, sensitivity and specificity to detect a significant CoA were determined. A significant CoA was defined as a ratio of the CoA diameter to the diaphragmatic aortic diameter of < 0.5 as imaged by MRI or angiography. Indexed systolic velocity and pressure half-times were found not to be significant predictors for CoA. For the Doppler parameter dVHTi, using a critical value of > 200 msec indexed, we found a positive predictive value of 87% and a negative predictive value of 80%. The parameter dPHTi, using a critical value of > 75 msec indexed, demonstrated positive and negative predictive values of 92% and 79%, respectively. Measurement of dVHTi is a useful predictor for significant CoA, but the parameter dPHTi has an improved positive predictive value for detection of significant CoA. Systolic measurements of velocity or pressure half-times are not adequate to assess severity of CoA.     

Mitral annular calcification and coarctation of aorta

Although coarctation of the aorta is associated with various cardiac abnormalities, its association with mitral annular calcification (MAC) is not reported. We therefore report a 37-year-old patient with postductal coarctation of the aorta and MAC. Considered to be a degenerative process, MAC is usually seen in elderly patients. It is uncommon in patients less than 50 years old and rare before the age of 40 years. Previous studies have reported increased frequency of cardiac conduction disturbances in patients with MAC compared to age- and sex-matched controls. This is in agreement with complete right bundle-branch block noted on the electrocardiogram of our patient. Clinicopathologic correlative studies and long-term follow-up may determine how such conduction disturbances develop and progress.     

Effective use of captopril in postoperative paradoxical hypertension of coarctation of the aorta

Severe systemic hypertension may complicate the postoperative course of patients undergoing surgical repair for coarctation of the aorta. The sympathetic and the renin-angiotensin systems have been demonstrated as underlying mechanisms for this paradoxical phenomenon (Farrell et al., 1979; Rocchini et al., 1979). We recently utilized captopril, a competitive inhibitor of angiotensin converting enzyme, with success in the management of severe hypertension that developed in a 15-year-old male after successful surgical repair for coarctation of the aorta. To our knowledge, this is the first report on the use of captopril for the treatment of this paradoxical phenomenon.     

Implantation of balloon-expandable stents for coarctation of the aorta: Implantation data and short-term results

We report the immediate results and the short-term follow-up in a group of selected patients with coarctation of the aorta who underwent endovascular stent implantation. Balloon-expandable stents were implanted in 6 patients (mean age 19.8 ± 5.1 years) with coarctation of the aorta (4 recurrent and 2 native) who underwent a total of 7 procedures (6 implantation and 1 further expansion). The systolic peak pressure gradient was decreased from 36.7 ± 16.9 to 13.3 ± 23.2 mm Hg (P < 0.005). There was a 66% increase in the mean coarctation diameter from 9.3 ± 1.7 to 15.6 ± 3.1 mm (P = 0.001) with the ratio of the coarctation to descending aorta diameter, measured at the level of the diaphragm, increasing from 0.49 ± 0.1 to 0.81 ± 0.2 (P < 0.005). The dilatation was successful in expanding the stent to an acceptable diameter in 5 of 6 patients. One patient underwent successful further expansion of a stent implanted 22 months previously. There were no immediate complications during balloon expansion and stent implantation. One patient suffered a femoral arterial bleed requiring surgical repair. There was one unrelated death. All patients were hypertensive (systolic blood pressure >140 mm Hg) prior to stent implantation. At mean follow-up of 8 months, 3 patients are normotensive. There was no recurrence of coarctation, aortic dissection, or aneurysm formation in the patients in whom stent implantation was successful. These findings indicate that balloon-expandable stent implantation for coarctation of the aorta in selected patients is a safe and effective alternative approach for relieving the obstruction with a low complication rate and no recoarctation at short-term follow-up. © 1996 Wiley-Liss, Inc.     

Endovascular stents for treatment of coarctation of the aorta: Acute results and follow‐up experience

Balloon angioplasty as treatment for coarctation of the aorta is increasingly performed. Endovascular stents have been proposed as a means of improving the efficacy and safety of the procedure. In this report, we describe one institution's immediate results and clinical follow‐up after implantation of endovascular stents. Retrospective analysis for endovascular stent placement for coarctation of the aorta between 1993 and 2002 was made. The immediate hemodynamic results and clinical follow‐up were reviewed. Thirty‐two patients underwent attempted stent placement for coarctation. Twenty‐three patients had postoperative recurrent coarctation and nine had native coarctation. The systolic gradient decreased from 31 to 1.8 mm Hg (P = 0.001) and the diameter was increased 8.1 to 13.5 mm (P–0.001). Mean follow‐up was 1.5 years. The mean follow‐up gradient as assessed by sphygomomanometry was 13.1 mm Hg. Eight patients underwent 10 successful further dilations. Complications included one stent migration and one aortic dissection. The use of stents as an adjunct to balloon angioplasty in selected patients with coarctation can be performed with low complication rates and provides excellent immediate relief of obstruction with promising follow‐up. Further dilation of these stents is possible. Long‐term follow‐up is warranted. Catheter Cardiovasc Interv 2004;62:499–505. © 2004 Wiley‐Liss, Inc.     

升-降主动脉旁路术治疗成人复杂主动脉缩窄

目的：探讨升-降主动脉人工血管旁路术治疗成人复杂主动脉缩窄的手术效果并总结其临床经验。方法：2015年10月和2018年7月,应用升主动脉-降主动脉人工血管转流术治疗成人复杂主动脉缩窄2例,均为男性;年龄分别未22岁和46岁。两例病人均经桡动脉、足背动脉穿刺测压,根据术前、术后,桡动脉、足背动脉平均压差变化评价手术效果。结果：术后均治愈出院。术前桡动脉足背动脉平均压差48mmHg和55mmHg;术后桡动脉足背动脉平均压差6mmHg和9mmHg,较术前明显缩小。术后主动脉CTA复查示转流人工血管通畅。结论：升-降主动脉人工血管旁路术是治疗成人复杂主动脉缩窄的有效手段。     

Endovascular stents in the management of coarctation of the aorta in the adolescent and adult: one year follow up

OBJECTIVES—To test the hypothesis that endovascular stents used with dilation of coarctation of the aorta (CoA) improve late outcomes. Balloon dilation for CoA has been limited by concerns over the risk for acute dissection, late restenosis, or aneurysm formation.
DESIGN—All patients seen with CoA between November 1994 and September 1997 underwent attempted stent implantation. Follow up was obtained for all patients and a subgroup (n = 18) had repeat catheterisation at a mean (SD) of 1.3 (0.5) years to assess residual gradient and stent-CoA morphology.
RESULTS—Stents were placed in 27 patients (15 male and 12 female patients, mean age 30.1 (13.1) years), of whom seven had prior surgical coarctectomy and one had a prior balloon dilation. Hypertension was present in 26 patients (mean pressure 164 (26)/86 (13) mm Hg), of whom 16 were on antihypertension drugs. CoA gradients were 46 (20) mm Hg (range 18-106 mm Hg) at baseline and 3 (5) mm Hg after the procedure. One patient had a stroke following the procedure; another patient had incomplete dilation and underwent a second procedure. At 1.8 (1) years after the procedure the mean pressure was 130 (14)/74 (11) mm Hg with seven patients on antihypertension treatment. The clinical gradient was 4 (8) mm Hg (range 0-32 mm Hg). At follow up angiography, the mean gradient was 4(6) mm Hg, and two patients had a gradient over 10 mm Hg. Aneurysms formed in three patients at the dilation site; one patient was referred for surgery.
CONCLUSION—In this age group stent management for CoA appears to be an effective technique and results in sustained reduction in CoA gradients at early term follow up, but aortic aneurysm was detected in 17% of patients who had repeat angiography.


Keywords: angioplasty; coarctation of the aorta; congenital heart defects; stents     

Long-term effects of balloon angioplasty on systemic hypertension in adolescent and adult patients with coarctation of the aorta.

AIMS: To define the long-term effect of balloon angioplasty of aortic coarctation on hypertension, in adolescent and adult patients. METHODS: Balloon angioplasty of discrete, native aortic coarctation was performed on 50 patients (34 male) aged 23+/-8 (mean+/-standard deviation) years. In 42 of these patients cardiac catheterization and angiography were repeated 1 year later, and on the basis of sphygmomanometric blood pressure determination at that time, they were divided into 31 patients (group A) with normalized blood pressure and 11 patients (group B) who still needed antihypertensive medication. Both groups were followed annually thereafter for 12-123 (66+/-37) months. RESULTS: Coarctation gradient values before, immediately after and 1 year after angioplasty were 69+/-24 mmHg, 12+/-8 mmHg (P<0.001) and 7+/-6 mmHg. The corresponding systolic blood pressure values were 165+/-17 mmHg, 128+/-12 mmHg (P<0.001) and 115+/-10 mmHg (P<0.001) in group A; 182+/-21 mmHg, 141+/-24 mmHg (P<0.001) and 134+/-18 mmHg (P<0.001) in group B. Echocardiographic left ventricular mass index before angioplasty and at follow-up was 130+/-31 g x m-2 and 105+/-23 g x m-2 in group A; 157+/-38 g x m-2 and 132+/-35 g x m-2 in group B (P<0.001 for both comparisons). CONCLUSION: Normalization of blood pressure without medication occurred in 74% of patients after angioplasty for aortic coarctation, with subsequent long-term regression of left ventricular hypertrophy. In comparison to reported surgical results, balloon angioplasty should be considered as first line treatment for native, discrete aortic coarctation in adolescent and adult patients.     

Cardiovascular risk factors in adults with coarctation of the aorta

Background: The aging patient with adult congenital heart disease (ACHD) facesthe risk of developing atherosclerotic disease. Patients with coarctation of the aorta(CoA) are especially vulnerable because of an inherent high risk of developing hy‐pertension. However, data on the prevalence of other cardiovascular risk factors arescarce. Therefore, this study aimed to describe the prevalence of traditional cardio‐vascular risk factors (diabetes, hypertension, hyperlipidemia, smoking, obesity, andsedentary lifestyle) in adult patients with CoA.
Methods: Patients with CoA who were registered at the ACHD clinic in Gothenburgwere asked to participate in a comprehensive cardiovascular risk assessment. Thisassessment included a glucose tolerance test, cholesterol profile, ambulatory bloodpressure measurements, and a lifestyle questionnaire.
Results: A total of 72 patients participated. The median age was 43.5 years and 58.3%were men. Sixty‐six (91.7%) patients had ≥one cardiovascular risk factor and 40.3%had ≥three risk factors. Three (4.2%) patients were newly diagnosed with diabetesor impaired glucose tolerance. More than half of the patients had hyperlipidemia(n = 42, 58.3%) and 35 patients (48.6%) were overweight or obese. Only three (4.2%)patients smoked regularly. Of the 60 patients who underwent 24‐hour ambulatoryblood pressure measurement, 33 (55.0%) were hypertensive. Of the 30 patients withknown hypertension only 9 (30.0%) had well‐controlled blood pressure on ambula‐tory blood pressure measurement.
Conclusions: Cardiovascular risk factors among patients with CoA are prevalent. Thismay indicate a need for more aggressive screening strategies of traditional risk fac‐tors to minimize the risk of these patients also developing atherosclerotic disease.     

Absence of the fetal right pulmonary artery complicated with coarctation of the aorta: Prenatal and postnatal diagnosis

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital cardiovascular malformation that can present as an isolated lesion or may be associated with other congenital heart malformations. Several studies have reported UAPA after birth. To our knowledge, the absence of the right pulmonary artery in the fetus has not been reported. Here, we report a rare case of fetal right pulmonary artery absence with aortic coarctation, which was confirmed by postpartum ultrasound and computed tomography angiography (CTA). Our case demonstrates that fetal echocardiography, especially the three‐vessel view, is beneficial for the prenatal diagnosis of pulmonary artery malformations.     

Predictive factors for residual hypertension following aortic coarctation stenting

Native coarctation of the aorta (CoA) and recoarctation (reCoA) after initial surgical repair are frequently associated with hypertension (HT). Most CoA cases are amenable to transcatheter balloon angioplasty with stent implantation; however, the impact of stenting on arterial blood pressure (BP) is variable. We carried out a retrospective study to identify the predictive factors for residual HT despite optimal endovascular treatment. Patients who had undergone stent implantation for native CoA or reCoA with a pressure gradient of >20 mm Hg between the upper and lower limbs, between 2007 and 2015, were included. The geometry and level of hypoplasia of the aortic arch were determined by non‐invasive imaging, and BP measurements were performed pre‐ and post‐procedure. Thirty consecutive patients (median age: 18.5 years; 76.7% male) were included. Twenty‐three patients had HT before the procedure and 14 (46.7%) had post‐procedural HT despite optimal endovascular treatment. Residual HT post‐stenting was associated with longer stent length and gothic arch geometry. Age and body mass index (BMI) were also associated with residual HT. The pathologic association of abnormal arch geometry and aortic stent placement may lead to a loss of aortic compliance that is further increased by high BMI and older age. Determination of a patient's aortic arch anatomy and clinical profile can assist in defining those at high risk of residual HT despite optimized isthmic stent implantation.     

A case report on endarteritis in a child with coarctation of aorta

Coarctation of aorta(CoA), complicated by endarteritis in a children is very rare. Here we present a case of endarteritis in an unoperated CoA in a four year old boy. CoA had been diagnosed in the referring hospital, yet the diagnosis of endocarditis distal to CoA, was made in the tertiary center using modified transthoracic echo windows or focused views. After six weeks of intravenous antibiotic treatment, a coarctectomy and end‐to‐end anastomosis was performed and he recovered clinically well. This case report concludes that echocardiography remains as the standard diagnostic method for identifying intracardiac manifestations of infective endocarditis/endarteritis. Last but foremost, it delineates the importance of modified transthoracic echo windows or focused views in identifying the unusual position of endocarditis.     

Long-term results (up to 18 years) of balloon angioplasty on systemic hypertension in adolescent and adult patients with coarctation of the aorta

BACKGROUND: Little is known regarding the long-term follow-up results of balloon angioplasty (BA) for patients with aortic coarctation (AC) on systemic hypertension. HYPOTHESIS: To define the long-term effect of BA of AC on systemic hypertension in adolescent and adult patients. METHODS: Follow-up data of 53 patients (36 male), mean age 24 +/- 9 years, undergoing BA for discrete AC at median interval of 11.8 years (range, 4-18 years), including cardiac catheterization, magnetic resonance imaging (MRI), and echocardiography. Patients were divided into two groups at 1 year after BA on the basis of absence (group A: 40 patients) or presence (group B: 11 patients) of persistent hypertension and need for medication. RESULTS: Fifty-one patients had baseline hypertension, BA produced an immediate reduction in peak AC gradient from 66 +/- 23 mmHg to 10.8 +/- 7 mmHg (p < 0.0001). The immediate systolic blood pressure (SBP) decreased from 165 +/- 17 mmHg (95% CI: 159 to 171) to 125 +/- 10 mmHg (95% CI: 122 to 131) (p < 0.001) in 40 patients (group A) and from 184 +/- 19 mmHg (95% CI: 169 to 198) to 142 +/- 22 mmHg (95% CI: 124 to 156) (p<0.001) in 11 patients (group B). At 1-year follow-up, SBP decreased further to 115 +/- 10 in group A (95% CI 111-119) and 134 +/- 19 in group B (95% CI 122-142) (p<0.001). The blood pressure had normalized without medication in group A (165 +/- 17 to 115 +/- 10 mmHg). CONCLUSION: Long-term results of BA for discrete AC are excellent and should be considered as the first option for treatment of this disease. No paradoxical hypertension occurred post angioplasty, and normalization of blood pressure without medication occurred in 78% of the patients after BA.     

Combined angioplasty and valvuloplasty for coexisting coarctation of the aorta and aortic stenosis in an infant

The patient was a 1-month-old male infant. Echocardiography showed left ventricular hypertrophy with coarctation of the aorta (CoA) and aortic valve stenosis (AVS). Left ventricular systolic function was poor. At catheterization, the gradient of AVS was 25 mmHg and the annulus size of the aortic valve was 7 mm. There was a CoA measuring 2.1 mm in diameter. Balloon valvuloplasty of the CoA was then performed using the same balloon; the pressure gradient decreased to 0 mmHg, and CoA diameter decreased from 2.1 to 5.5 mm. Cathet. Cardiovasc. Diagn. 45:51–53, 1998. © 1998 Wiley-Liss, Inc.     

Stenting vs. balloon angioplasty for discrete unoperated coarctation of the aorta in adolescents and adults.

More information is needed to clarify whether stenting is superior to balloon angioplasty (BA) for unoperated coarctation of the aorta (CoA). From September 1997, 21 consecutive adolescents and adults (24 +/- 11 years) with discrete CoA underwent stenting (G1). The results were compared to those achieved by BA performed in historical group of 15 patients (18 +/- 10 years; P = 0.103; G2). After the procedure, systolic gradient reduction was higher (99% +/- 2% vs. 87% +/- 17%; P = 0.015), residual gradients lower (0.4 +/- 1.4 vs. 5.9 +/- 7.9 mm Hg; P = 0.019), gain at the CoA site higher (333% +/- 172% vs. 190% +/- 104%; P = 0.007), and CoA diameter larger (16.9 +/- 2.9 vs. 12.9 +/- 3.2 mm; P < 0.001) in G1. Aortic wall abnormalities were found in eight patients in G2 (53%) and in one in G1 (7%; P < 0.001). There was no major complication. Repeat catheterization (n = 33) and/or MRI (n = 2) was performed at a median follow-up of 1.0 year for G1 and 1.5 for G2 (P = 0.005). Gradient reduction persisted in both groups, although higher late gradients were seen in G2 (median of 0 mm Hg for G1 vs. 3 for G2; P = 0.014). CoA diameter showed no late loss in G1 and a late gain in G2 with a trend to being larger in G1 (16.7 +/- 2.9 vs. 14.6 +/- 3.9 mm; P = 0.075). Two patients required late stenting due to aneurysm formation or stent fracture in G1. Aortic wall abnormalities did not progress and one patient required redilation in G2. Blood pressure was similar in both groups at follow-up (126 +/- 12/81 +/- 11 for G1 vs. 120 +/- 15/80 +/- 10 mm Hg for G2; P = 0.149 and 0.975, respectively). Although satisfactory and similar clinical outcomes were observed with both techniques, stenting was a better means to relieve the stenosis and minimize the risk of developing immediate aortic wall abnormalities.     

Influence of the postoperative period and surgical procedure on ambulatory blood pressure-determination of hypertension load after successful surgical repair of coarctation of the aorta

Aims This study quantified hypertension load using 24-h ambulatoryblood pressure monitoring after successful repair of coarctationof the aorta less than (1) or more than 10 years previously(2) and examined the influence of the surgical procedure (anastomosisor subclavian flap). Methods and Results Ambulatory blood pressure recordings were obtained using anAccutracker II monitor every 30min during the day and hourly,at night. Day and night systolic and diastolic values were higherin coarctation of the aorta than in controls: (day: systolicblood pressure/diastolic blood pressure: 133/71±6/4 vs115/66±3/2 night: systolic blood pressure/diastolic bloodpressure: 117/61±4/4 vs 107/57±3/2mmHg, P<0·01)and at all times, were higher in coarctation of the aorta (2)than in coarctation of the aorta (1). Clinical daytime systolichypertension was observed in 20% of recordings from coarctationof the aorta (1) and 49% from coarctation of the aorta (2) whilediastolic hypertension was not observed. However, systolic bloodpressure and diastolic blood pressure responses to daily activitieswere significantly higher in coarctation of the aorta than incontrols and this was more marked in coarctation of the aorta(2) than in coarctation of the aorta (1). Type of surgery didnot affect either hypertension prevalence or blood pressurereactivity. Conclusions These observations indicate exaggerated systolic blood pressureand diastolic blood pressure reactivity after repair of coarctationof the aorta, the prevalence of systolic hypertension doubling10 years after surgery.