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Author(s): Skaiste Sendzikaite [sup.1] , Rita Sudikiene [sup.1] , Virgilijus Tarutis [sup.1] , Inguna Lubaua [sup.2] , Pauls Silis [sup.2] , Agata Rybak [sup.3] , Augustina Jankauskiene [sup.1] , Mieczyslaw [...], Background This study aimed to evaluate hemodynamic phenotypes and prevalence of left ventricular hypertrophy in children after coarctation repair with right arm and leg blood pressure difference < 20 mmHg. Secondary objectives were analysis of effects of age at intervention, residual gradient across the descending aorta, and type of correction. Methods Blood pressure status and left ventricular hypertrophy were diagnosed according to European Society of Hypertension 2016 guidelines. Results Of 90 patients with a median age 12.5 (8.9-15.8) years, 8.5 (6.0-11.8) years after coarctation repair who were included, 42 (46.7%) were hypertensive. Isolated systolic hypertension dominated among 29 hypertensive patients with uncontrolled or masked hypertension (25 of 29; 86.2%). Of the 48 patients with office normotension, 14.6% (7) had masked hypertension, 8.3% (4) had ambulatory prehypertension, and 54.2% (26) were truly normotensive. Left ventricular hypertrophy was diagnosed in 29 patients (32.2%), including 14 of 42 (33.3%) hypertensive and 15 of 48 (31.3%) normotensive patients. The peak systolic gradient across the descending aorta was greater in hypertensive subjects (33.3 ± 12.7 mmHg) compared with normotensive subjects (25 ± 8.2 mmHg, p = 0.0008). Surgical correction was performed earlier than percutaneous intervention (p < 0.0001) and dominated in 40 of 48 (83.3%) normotensive versus 24 of 42 (57.1%) hypertensive patients (p = 0.006). Conclusions Arterial hypertension with isolated systolic hypertension as the dominant phenotype and left ventricular hypertrophy are prevalent even after successful coarctation repair. Coarctation correction from the age of 9 years and older was associated with a higher prevalence of hypertension.

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.ahj.2007.03.046 Byline: Josep Rodes-Cabau (a), Joaquim Miro (b), Adrian Dancea (c), Reda Ibrahim (d), Eric Piette (e), Chantale Lapierre (b), Luc Jutras (c), Jean Perron (a), Christo I. Tchervenkow (c), Nancy Poirier (b), Nagib S. Dahdah (b), Christine Houde (f) Abstract: The objectives of this study were to compare, in the current era, the immediate results, complications, and midterm outcomes obtained by surgical repair versus transcatheter treatment of coarctation of the aorta (CoA). Author Affiliation: (a) Quebec Heart Institute-Laval Hospital, Quebec, Quebec, Canada (b) Sainte-Justine Hospital, Montreal, Canada (c) Children's Hospital, Montreal, Canada (d) Montreal Heart Institute, Montreal, Canada (e) Montreal University, Montreal, Canada (f) Laval University Hospital, Quebec, Quebec, Canada Article History: Received 20 January 2007; Accepted 28 March 2007 Article Note: (footnote) The study was supported in part by a grant from the Fondation de l'Institut de Cardiologie de Quebec and the Reseau en Sante Cardiovasculaire du Fonds de Recherche en Sante du Quebec.

Abstract. Different percutaneous interventions can be used to treat coarctation of the aorta. However, a great amount of information is still needed regarding the long-term course. This article reviews our [...]

Byline: J. C. George (1), D. Shim (1), J. C. Bucuvalas (1), E. Immerman (1), P. B. Manning (1), J. M. Pearl (1), R. H. Beekman (1) Keywords: Coarctation; Stent; Congenital heart disease; Costs Abstract: The cost-effectiveness of stent (ST) implantation for the repair of coarctation of the aorta (CoA) is not documented in the medical literature. Inflation-adjusted hospital costs for ST implantation and for surgical (SU) repair were obtained using the HBOC Cost Accounting System software and evaluated for all patients 5 years of age or older who underwent elective treatment of CoA between July 1997 and June 2001. The average age of the ST group (n = 10) was 12.2 +- 3.9 years compared to 9.5 +- 3.5 years for the SU group (n = 12) (p &gt 0.10). The ST group had one failure due to inability to cross the CoA (failure rate, 10%). Successful repair was accomplished in all other ST cases and in all SU cases, with no residual systolic gradients at 1-year follow-up. Hospital length of stay for the ST group was 0.8 +- 1.2 days compared to 3.5 +- 0.5 days for the SU group (p &lt 0.001). The mean inflation-adjusted cost for the ST group was $7,148 +- 2,984 versus $11,769 +- 3,702 for the SU group (p &lt 0.005). By intention to treat analysis, the cost of repair in the ST-first group was $8,325 +- 3,354 given the 10% failure rate (p &lt 0.04 vs the SU only group). Sensitivity analysis demonstrates that cost of repair is lower with the ST-first strategy compared to SU only until the failure rate of ST implantation exceeds 39%. Repair of CoA using an endovascular stent strategy is cost-effective compared to conventional surgical repair. Author Affiliation: (1) The Heart Center, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229-3039, USA Article History: Registration Date: 01/01/2003 Online Date: 29/07/2003

Byline: A. Al-Hroob (), T.S. Husayni (), A. Freter (), P. Chiemmongkoltip (), M.N. Ilbawi (), R.A. Arcilla () Abstract: Aortic aneurysm may develop after surgery for coarctation of aorta especially patch aortoplasty. The size of patch and of adjacent native aortic wall was analyzed to determine whether aortic dilatation represents a true aneurysm. Electron beam tomography (EBT) was done on 19 patients, three months to 17.5 years after patch aortoplasty. Tomograms of aorta were obtained in 6-mm slices, and maximal cross-sectional area was digitized to obtain: total circumference (Ct), patch component (Cp), and aortic wall component (Cw). Ct, Cp, and Cw were normalized to the circumference of distal aorta (Cda) as: isthmus/distal aorta (Ct/Cda), patch segment/distal aorta (Cp/Cda), wall segment/distal aorta (Cw/Cda). Ct/Cda ranged from 109% to 260%. In 12 patients (group A), it varied from 168% to 260% and in seven (group B), 109% to 133%. There was strong correlation (r = 0.92) between Ct/Cda and Cp/Cda. Ct/Cda, Cp/Cda, and Cp/Cw were higher in group A than B (p &lt 0.001) but Cw/Cda did not differ. Cw/Cda was greater than the coarctation/distal aorta diameter ratios of preoperative angiograms, consistent with accelerated aortic wall growth postsurgery. No definite aneurysm was seen. Localized dilatation of aorta following patch aortoplasty in children is primarily due to a large synthetic patch and, partly, to increased aortic wall growth. Serial EBT or magnetic resonance imaging is indicated to monitor aortic wall growth and occurrence of aneurysm. Author Affiliation: () Divisions of Cardiology and Cardiac Surgery, The Heart Institute for Children, Hope Children's Hospital, Advocate Christ Medical Center, Oak Lawn, IL, US () University of Illinois at Chicago, Chicago, IL, US Article History: Received Date: 24/09/2001 Accepted Date: 07/03/2002

Byline: H.T. Patel (1), A. Madani (1), Y.M. Paris (1), K.G. Warner (1), Z.M. Hijazi (1) Keywords: Key words: Native coarctation -- Balloon angioplasty -- Neonate -- Infant Abstract: Balloon angioplasty (BA) for native coarctation of the aorta (CA) in infants and neonates remains controversial with a high incidence of restenosis. The purpose of this study is to analyze our acute and midterm results for BA of native CA in infants and neonates and try to identify factors that may be predictive of outcome. Between September 1991 and June 1999, 17 patients with CA underwent BA at a median age of 3 months (range 2 weeks--9 months) and median weight of 4.8 kg (range 2.8--7 kg). Fourteen patients had discrete CA and 3 had tubular hypoplasia. All patients were hemodynamically stable prior to BA and no patients had critical coarctation requiring prostaglandin E.sub.1 infusion to maintain ductus arteriosus patency. Seven patients had other associated cardiac defects. All patients had significant initial improvement. The mean peak systolic gradient across the CA improved from 43 +- 15 mmHg to 10 +- 8 mmHg (p &lt 0.001), and the mean minimum diameter of the aortic lumen increased from 2.4 +- 0.9 mm to 5.2 +- 1.0 mm (p &lt 0.001). There was no mortality or major complication. At median follow-up interval of 2.7 years (0.15--7.75 years), 10 (59%) of 17 patients are clinically well and have an upper to lower limb systolic blood pressure difference of &lt 20 mmHg. Seven (41%) of 17 patients developed significant restenosis (5 of these patients underwent repeat BA, which was successful in 3 patients). Four (24%) patients underwent surgical repair at a median age of 4.5 months (3--6.9 months) and a median time interval of 4 months (2--6.5 months) from the initial BA. All 3 patients with tubular hypoplasia type of CA underwent surgical repair. No patients developed aortic aneurysm following initial or repeat BA. All patients who underwent surgical repair were 1 month or less in age at the time of their initial BA. We conclude that BA of native CA in infants and neonates can be performed safely with low mortality and morbidity. It appears to offer the best results in patients who are older than 1 month with discrete CA and a well-developed aortic arch. Further restenosis of the discrete CA can be managed successfully by repeat BA. Author Affiliation: (1) Division of Pediatric Cardiology, Department of Pediatrics, Floating Hospital for Children at New England Medical Center, Tufts University School of Medicine, Boston, MA, USA, US

Byline: H. Senzaki (1), T. Kobayashi (1), K. Koike (1), N. Sasaki (1) Keywords: Key words: Stent -- Coarctation of the arota -- Complication Abstract: Recent studies have shown that stenting for coarctation of the aorta (CoA) may be an effective treatment modality for this disease. We report a case of stent repair of CoA in a 17-year-old patient. An incompletely expanded stent caused by a balloon rupture had been implanted in the femoral artery. Subsequent implantation for CoA was successfully achieved using a high-profile balloon catheter. Stent implantation may be an effective modality for CoA repair and may prove useful as an initial procedure for adolescents and adults with CoA. However, one must consider that incomplete expansion of a stent associated with dilatation balloon rupture can cause serious complications. The current case raises important issues that require further discussion to improve the results of this procedure. Author Affiliation: (1) Division of Pediatric Cardiology, Department of Pediatrics, Saitama Medical School Hospital, 38 Morohongo, Moroyama, Iruma-Gun, Saitama, 350 Japan, JP

Coarctation of the aorta (CoA) is defined as a congenital stenosis of the thoracic aorta and is one of the most common congenital cardiovascular diseases. Despite successful surgical treatment for CoA, arterial abnormalities, including refractory hypertension, aortic aneurysm, and proatherogenic phenotypic changes, frequently affect patients' quality of life. Emerging evidence from morphological and molecular biological investigations suggest that the area of CoA is characterized by phenotypic modulation of smooth muscle cells, intimal thickening, and impaired elastic fiber formation. These changes extend to the pre-and post-stenotic aorta and impair arterial elasticity. The aim of this review is to present current findings on the pathology and molecular mechanisms of vascular remodeling due to CoA. In particular, we will discuss the association between CoA and the ductus arteriosus since the most common site for the stenosis is in the proximity of the ductus arteriosus. Keywords: Congenital heart disease, Coarctation of the aorta, Remodeling, Ductus arteriosus, Author(s): Utako Yokoyama[sup.1], Yasuhiro Ichikawa[sup.1], Susumu Minamisawa[sup.2] and Yoshihiro Ishikawa[sup.1] Introduction Early diagnosis and appropriate management of coarctation of the aorta (CoA) have resulted in a low mortality rate associated [...]

Byline: Jinping Xu, Takahiro Shiota, Ryozo Omoto, Xiaodong Zhou, Shunei Kyo, Masahiro Ishii, Mary J. Rice, David J. Sahn Abstract: Background: Impaired aortic pulsatility has been demonstrated by angiography in children and in studies of experimental animals with coarctation of the aorta. Objectives: The purpose of this study was to assess regional aortic stiffness, distensibility, and compliance before and after balloon dilation in patients with coarctation of the aorta. Methods and Results: Intravascular ultrasound examination was performed in 13 pediatric patients with the diagnosis of coarctation of the aorta to yield aortic diameter. Area transverse sections at both systolic and diastolic period were measured at three aortic levels: the proximal, distal, and coarctation segments. Balloon dilation was also performed in eight of 13 patients. By using pressures measured in the same areas, an aortic stiffness index ([beta]) was calculated as ln(Ps/Pd)/(Ds-Dd), where ln is natural logarithm, Ps is systolic pressure, Pd is diastolic pressure, Ds is systolic diameter, and Dd is diastolic diameter. Aortic distensibility and an estimation of aortic compliance were also calculated. The [beta] stiffness index of the coarctation and the proximal segments of the aorta were significantly greater than that of the distal segment of the aorta (p < 0.01). The aortic wall stiffness [beta] index did not acutely change after successful balloon dilation, but the distensibility and compliance of distal aorta were nonetheless significantly decreased after balloon dilation (p < 0.01, p < 0.05) as a function of changes of pulsatility of flow. Conclusions: Abnormal proximal aortic stiffness may be a strong contributing factor that promotes the genesis of hypertension in patients with coarctation even after successful repair or balloon angioplasty. (Am Heart J 1997;134:93-98.) Author Affiliation: Portland, Ore., and Saitama, Japan Article History: Received 5 August 1996; Accepted 9 April 1997 Article Note: (footnote) [star] From the Clinical Care Center for Congenital Heart Disease, Oregon Health Sciences University, and Saitama Medical School., [star][star] Reprint requests: David J. Sahn, MD, The Clinical Care Center for Congenital Heart Disease, Oregon Health Sciences University, 3181 S.W. Sam Jackson Park Rd., UHN-60, Portland, OR 97201-3098., a E-mail: sahnd@ohsu.edu, aa 0002-8703/97/$5.00 + 0 4/1/82545

Coarctation of the aorta is a not uncommon congenital defect in which the main systemic artery, the aorta, is constricted after leaving the heart. This constriction, of course, increases the work the heart must perform to force the blood through the aorta. The defect can be surgically corrected in children, but evidence is accumulating that even in cases of apparently successful repair, heart function does not return to normal. Previous studies have shown that the hypertrophy (enlargement) of the left ventricle that results from the additional workload of the coarctation persists for decades after the coarctation is repaired. Echocardiographic examination of 11 patients and 22 age-matched controls revealed greater fractional shortening and greater peak shortening velocity (measurements of changes in the size of the ventricles during contraction) were observed in the coarctation patients. In keeping with previous studies, greater left ventricular mass was observed. Abnormalities were observed in the diastolic filling of the ventricle. The LV mass and the LV fractional shortening were found to be correlated with the small residual arm-leg gradient in blood pressure (the difference in blood pressure taken at the leg and the arm). The authors speculate that this small arm-leg gradient in blood pressure is indicative of underlying abnormalities in circulation which may be responsible for the persistent heart abnormalities in these patients. The clinical implications of these persistent heart abnormalities are not known. (Consumer Summary produced by Reliance Medical Information, Inc.)

Byline: Y. Isomatsu (1), Y. Takanashi (1), M. Terada (1), K. Kasama (1) Keywords: Persistent fifth aortic arch; Aortic coarctation; Aortic interruption Abstract: A 28-year-old woman having persistent fifth aortic arch and aortic coarctation associated with the fourth arch interruption, without other cardiac malformation, underwent graft interposition, 20 mm in diameter, between the fourth arch including the left subclavian artery and the descending aorta. Author Affiliation: (1) Division of Cardiovascular Surgery, Department of Surgery, Yokohama City University School of Medicine, Yokohama, Japan Article History: Registration Date: 01/01/2003 Online Date: 28/01/2004