Rare variants in NR2F2 cause congenital heart defects in humans.

Congenital heart defects (CHDs) are the most common birth defect worldwide and are a leading cause of neonatal mortality. Nonsyndromic atrioventricular septal defects (AVSDs) are an important subtype of CHDs for which the genetic architecture is poorly understood. We performed exome sequencing in 13 parent-offspring trios and 112 unrelated individuals with nonsyndromic AVSDs and identified five rare missense variants (two of which arose de novo) in the highly conserved gene NR2F2, a very significant enrichment (p = 7.7 × 10(-7)) compared to 5,194 control subjects. We identified three additional CHD-affected families with other variants in NR2F2 including a de novo balanced chromosomal translocation, a de novo substitution disrupting a splice donor site, and a 3 bp duplication that cosegregated in a multiplex family. NR2F2 encodes a pleiotropic developmental transcription factor, and decreased dosage of NR2F2 in mice has been shown to result in abnormal development of atrioventricular septa. Via luciferase assays, we showed that all six coding sequence variants observed in individuals significantly alter the activity of NR2F2 on target promoters.

Anatomical considerations for the management of a left-sided superior caval vein draining to the roof of the left atrium.

Aims The pathophysiological entity of a persisting left-sided superior caval vein draining into the roof of the left atrium represents an extreme form of coronary sinus de-roofing. This is an uncommon, but well-documented condition associated with systemic desaturation due to a right-to-left shunt. Depending on the size of the coronary ostium, the defect may also present with right-sided volume loading. We describe two patients, both of whom presented with desaturation, and highlight the important anatomical features underscoring management. Methods and Results Both patients were managed interventionally with previous assessment of the size of the coronary sinus ostium through cross-sectional imaging. This revealed a restrictive interatrial communication at the right atrial mouth of the coronary sinus in both patients, which permitted an interventional approach, as the residual left-to-right shunt subsequent to closure of the aberrant vessel would be negligible. At intervention, test occlusion of the left superior caval vein allowed assessment of decompressing vessels before successful occlusion using an Amplatzer Vascular Plug. CONCLUSIONS: Persistence of a left superior caval vein draining to the left atrium may be associated with an interatrial communication at the mouth of the unroofed coronary sinus. The ostium of the de-roofed coronary sinus can be atretic, restrictive, normally sized, or enlarged. Careful assessment of the size of this defect is required before treatment. In view of its importance, which has received little attention in the literature to date, we suggest an additional consideration to the classification of unroofed coronary sinus.

Implementing transition: Ready Steady Go.

There is good evidence that morbidity and mortality increase for young persons (YP) following the move from paediatric to adult services. Studies show that effective transition between paediatric and adult care improves long-term outcomes. Many of the issues faced by young people across subspecialties with a long-term condition are generic. This article sets out some of the obstacles that have delayed the implementation of effective transition. It reports on a successful generic transition programme 'Ready Steady Go' that has been implemented within a large National Health Service teaching hospital in the UK, with secondary and tertiary paediatric services, where it is now established as part of routine care.

Midterm evaluation of biological prosthetic valves in the pulmonary position of grown-up patients.

OBJECTIVES: To examine the midterm clinical outcome of pulmonary valve replacement (PVR) with prosthetic valves. METHODS: We reviewed 37 consecutive patients who underwent PVR with biological prosthetic valves between September 1999 and June 2010. The median age was 22.6 years (range: 6 to 70 years; three children). The primary diagnosis was Tetralogy of Fallot in 20 patients (54%). Valve pathology was regurgitation in 27 patients (72.9%). Cardiac surgery had been previously performed in 35 patients (94.5%). The median size of the prosthesis was 25 mm (range: 21 to 31 mm). The median follow-up was 42 months (range: 1.2 to 129 months). RESULTS: There were no early valve-related deaths. Hospital mortality was 2.7% (n = 1) and no patient required early rereplacement of prosthesis. Two patients required permanent pacemaker insertion. During follow-up, there was no late death, reoperation for structural valve degeneration, or valve thrombosis. Only one patient required repeated operation for endocarditis at 37 months follow-up. The actuarial survival at 5 years was 95.1 ± 3.8%. Overall freedom from reoperation after PVR at 5 years was 93.0 ± 8.6%. At last follow-up, 34 patients (91.8%) were NYHA class I versus 20 patients (54%) preoperatively (p < 0.05). In the 35 surviving patients who did not undergo redo-PVR, there was no-mild regurgitation and the peak PV gradient was 20.4 ± 10.2 mm Hg (16.2 ± 9.3 mm Hg preoperatively). Thirty-two patients (91.4%) had good right ventricular function compared with 26 patients (74.2%) preoperatively. CONCLUSIONS: PVR with biological prosthetic valves can be performed with good midterm survival, functional status, and haemodynamics.

Familial Ebstein Anomaly: Whole Exome Sequencing Identifies Novel Phenotype Associated With FLNA.

BACKGROUND: Familial Ebstein anomaly is a rare form of congenital heart disease. We report 7 individuals among 2 generations of 1 family with Ebstein anomaly. This family was first reported in 1991 by Balaji et al in which family members were also reported to have a mild skeletal phenotype. The most likely mechanism of inheritance was concluded to be autosomal dominant. We sought to identify the genetic pathogenesis in this family using a next generation sequencing approach. METHODS AND RESULTS: Whole exome sequencing was performed in 2 cousins in this family using the Agilent SureSelect Human all Exon 51 Mb version 5 capture kit. Data were processed through an analytic in-house pipeline. Whole exome sequencing identified a missense mutation in FLNA (Filamin A), an actin-binding protein located at Xq28, mutations in which are associated with the skeletal phenotypes Frontometaphyseal dysplasia, Otopalatodigital, and Melnick-Needles syndrome, with X-linked periventricular nodular heterotopia and FG syndrome (Omim, 305450). Review of the phenotypes of those with the mutation in this family shows increased severity of the cardiac phenotype and associated skeletal features in affected males, consistent with X-linked inheritance. CONCLUSIONS: Although congenital heart disease is reported in families with mutations in FLNA, this is the first report of individuals being affected by Ebstein anomaly because of a mutation in this gene and details the concurrent skeletal phenotype observed in this family.

Medium term outcome for infant repair in tetralogy of Fallot: Indicators for timing of surgery.

OBJECTIVE: To assess the impact of early corrective surgery on the short and medium term outcome in tetralogy of Fallot (TOF). MATERIALS AND METHODS: All patients under 12 months of age undergoing correction of isolated TOF between February 1997 and July 2003 were reviewed retrospectively. Outcome data for mortality, post-operative care management, major morbidity and clinical follow-up were analysed. RESULTS: Fifty-two operations were performed. The mean age at surgery was 5 months (range 1-12) of whom 16 (30.8%) were less than 3 months old, including 2 neonates, 22 (42.3%) were 3-6 months old and 14 (26.9%) were 7-12 months old. There was 1 (1.9%) early death caused by a cerebro-vascular accident and 1 (1.9%) late death secondary to acute infective endocarditis. There were no differences in post-operative morbidities attributable to age. Patients under 3 months old required greater duration of post-operative ventilation, ITU stay and in-hospital stay. At a mean follow-up of 4.0 years (range 1.5-8.0), 33 (63.5%) patients had well-tolerated pulmonary regurgitation (PR) and 3 (5.8%) patients required re-operation for right ventricular outflow tract obstruction (RVOTO). All patients had right bundle-branch-block but with QRS < 150 ms. CONCLUSION: Early definitive repair of TOF can be performed safely on patients under 6 months old. Age at surgery does not appear to affect the medium term haemodynamic outcome. However, early surgery does escalate the need for ICU care. This data suggests repair in asymptomatic patients be delayed until 3-6 months of age.

Reoperations and survival after primary repair of congenital heart defects in children.

OBJECTIVE: The objective of this article is to evaluate the incidence of reoperation and the associated risk and survival after primary repair of congenital heart defects using cardiopulmonary bypass in children. METHODS: We present a retrospective analysis of 1220 consecutive children under 16 years [649 (53%) under 1 year] operated on between 1976 and 2001 by 1 surgeon (J.L.M.). RESULTS: The early (30-day) mortality was 6.9%; 171 patients had 206 reoperations. The early mortality for the first reoperation was 10.4% and for second reoperation, 3.8%. Of the first-time reoperations 63% were inevitable, 15% were planned, and 22% were unexpected. The overall 20-year freedom from reoperation was 83% and survival (including early mortality) was 86%. The 10-year freedom from reoperation and survival, respectively, was as follows: aortic valvotomy, 77% and 92%; pulmonary valvotomy, 90% and 95%; atrial septal defect, 99% and 100%; partial atrioventricular septal defect (AVSD), 81% and 94%; complete AVSD, 74% and 70%; VSD, 95% and 97%; double-outlet right ventricle, 66% and 68%; truncus arteriosus, 54% and 71%; Mustard, 85% and 86%; arterial switch, 78% and 74%; Fontan, 77% and 66%; Fallot, 91% and 93%; and total anomalous pulmonary venous drainage, 89% and 84%. Of those undergoing aortic valvotomy, 53% were infants, but when aortic valve replacement became necessary an adult valve could be inserted. Introduction of the total cavopulmonary connection to the Fontan procedure in 1990 with prior cavopulmonary anastomoses has greatly improved outcome, with only 1 reoperation and no deaths since then. Patients with AVSD required 12 reoperations for mitral regurgitation, mostly through the "cleft," the closure of which in the past 5 years has provided promising results. CONCLUSIONS: The majority of reoperations after repair of congenital heart defects in children are inevitable, and their incidence varies for different types of procedures. These findings will help in informing parents about the possible outcome of surgery, but the introduction of newer techniques may reduce the need for reoperation further. The survival is encouraging.

Obstructed hemianomalous pulmonary venous drainage: is intervention necessary?

Obstructed infradiaphragmatic hemianomalous pulmonary venous drainage is associated with high mortality and morbidity, and treatment is best tailored to the individual patient. Resolution of an obstructed scimitar vein through collateralization has rarely been reported. We report two such cases and review the literature.

Repair of tetralogy of Fallot in infancy with a transventricular or a transatrial approach.

OBJECTIVE: The optimal time and approach of repair of tetralogy of Fallot (TOF) remain controversial. The purpose of this study was to evaluate the outcome following repair of TOF in infants with particular regard to the surgical approach used. PATIENTS: One hundred and sixty infants (mean age 195+/-89 days, range 11-364 days) undergoing repair of a simple TOF were studied. Between 1974 and 2000, a transventricular approach (RV) was used in 91 and between 1988 and 2000, a transatrial (RA) approach in 69 infants. Ten of these infants (6.2%) had a previous palliative shunt (four in the RV versus six in the RA group). A transannular patch (TAP) was inserted in 96 (60%) infants (76 versus 20). Follow-up was complete (mean 14.5+/-5.2 versus 6+/-1 years). RESULTS: There were three operative deaths (1.9%), (two in RV versus one in RA group). A re-operation for right ventricular outflow tract obstruction (RVOTO) was performed in 19 patients (3 versus 16). Ten-year freedom from re-operation for RVOTO (+/-standard error of the mean) was 88+/-4% (98+/-2 versus 72+/-6%, P<0.0001). Within the RA group, 5-year freedom from re-operation for RVOTO for those who had a TAP was 79+/-9% and it was 75+/-4% for those having a simple repair. Six patients in the RV group required pulmonary valve replacement (PVR). Ten-year freedom from PVR was 98+/-1% (97+/-2 versus 100%, P=0.3). There were two late deaths, one in each group. Ten-year survival was 97+/-1%. One patient in the RV group developed late recurrent ventricular tachycardia requiring the implantation of a defibrillator. At most recent echocardiography, all but the patient who had the defibrillator had good right and left ventricular function. CONCLUSIONS: Transventricular and transatrial repair of TOF in infancy, are associated with an acceptable operative risk, low incidence of late arrhythmia, good bi-ventricular function and excellent survival. In our experience, however, transatrial repair has a disturbing incidence of early and mid-term residual or recurrent RVOTO, even when a TAP has been used.

Defects in the oval fossa: morphologic variations and impact on transcatheter closure.

BACKGROUND: Incomplete formation of the partition between the two atrial chambers in the region of the oval fossa results in a range of defects, which extend from patent foramen ovale to large secundum atrial septal defects (ASDs). There is wide variation in the morphology of the latter lesions. The spatial orientation of the margins of ASDs relative to the persisting flap valve is not easily definable with standard echocardiographic imaging. Careful evaluation of the morphology is essential in optimizing successful transcatheter closure to minimize complications. The advent of three-dimensional transesophageal echocardiography has changed the understanding of the morphology of these defects and facilitated successful percutaneous closure. METHODS: Since 2007, over a 4-year period, transcatheter closure of ASDs was performed in 105 patients. RESULTS: During the study period, there were two instances of embolization of the device. The morphology of the defects in the patients with embolization was evaluated carefully, and an unusual spiral configuration of the flap valve relative to the rims of the oval fossa was noted. These findings were then found in four additional patients and serve as the focus of this report. To facilitate understanding of the unusual morphology, the clinical findings were compared with images showing the mechanism of development of the atrial septum in the mouse, revealing a striking similarity. CONCLUSIONS: Although uncommon, spiral spatial orientation of the margins of ASDs predisposes to embolization of devices used for percutaneous closure. Standard cross-sectional techniques have limited use in identifying this variation. Understanding of the development of the atrial septum in the mouse heart may help explain the morphogenesis of the defect and the mechanism predisposing to embolization.

Arterialised hepatic nodules in the Fontan circulation: hepatico-cardiac interactions.

Hypervascular nodules occur commonly when there is hepatic venous outlet obstruction. Their nature and determinants in the Fontan circulation is poorly understood. We reviewed the records of 27 consecutive Fontan patients who had computerized tomography scan (CT) over a 4 year period for arterialised nodules and alterations in hepatic flow patterns during contrast enhanced CT scans and related these findings to cardiac characteristics. Mean patient age was 24 ± 5.8 years, (range 16.7-39.8) and mean Fontan duration was 16.8 ± 4.8 years (range 7.3-28.7). Twenty-two patients demonstrated a reticular pattern of enhancement, 4 a zonal pattern and only 1 demonstrated normal enhancement pattern. Seven (26%) patients had a median of 4 (range 1-22) arterialised nodules, mean size 1.8 cm (range 0.5 to 3.2 cm). All nodules were located in the liver periphery, their outer aspect lying within 2 cm of the liver margin. Patients with nodules had higher mean RA pressures (18 mmHg ± 5.6 vs. 13 mmHg ± 4, p=0.025), whereas their mixed venous saturation and aortic saturation was not significantly different (70% ± 11 vs. 67% ± 9 and 92% ± 10 vs. 94% ± 4, p>0.05). Post-mortem histology suggests focal nodular hyperplasia is the underlying pathology. ConclusionsAbnormalities of hepatic blood flow and the presence of arterialised nodules are common in the failing Fontan circulation. They occur especially when central venous pressures are high, and very likely indicate arterialisation of hepatic blood flow and reciprocal portal venous deprivation. The underlying pathology is most likely focal nodular hyperplasia.

Primary biventricular repair of atrioventricular septal defects: an analysis of reoperations.

BACKGROUND: The purpose of this study was to analyze the factors affecting reoperation after primary biventricular atrioventricular septal defect (AVSD) repair. METHODS: Between April 1997 and April 2007, 93 consecutive patients underwent surgery for biventricular correction of AVSD with a median age of 5.8 months (range, 9 days to 68.9 years). Fifty-three patients had complete AVSD, 6 patients had an intermediate type, and 29 patients had partial AVSD; 4 patients had a complete AVSD with associated tetralogy of Fallot, and 1 patient had a complete AVSD with double-outlet right ventricle. RESULTS: There was no in-hospital mortality. There were 2 late deaths (2.2%). Forty-three reoperations were performed in 23 patients (24.7%), of which 18 were for repair of significant left atrioventricular valve regurgitation and 8 were mitral valve replacements. Seven patients (7.5%) required insertion of a permanent pacemaker. The overall 5-year freedom from reoperation after AVSD repair was 73.6% +/- 4.8%. In the multivariate analysis for complete AVSDs, Down syndrome (p = 0.01) and the presence of right ventricular dominance (p = 0.03) were independent predictors of reoperation. At last follow-up, 76 patients (83.5%) were in New York Heart Association class I, and 68 patients (74.7%) were not taking any heart failure medications. Echocardiographic examination showed absent to mild left atrioventricular valve regurgitation in 76.5%; moderate, in 19.8%; and severe, in 3.7% of patients. CONCLUSIONS: Down syndrome and right ventricular dominance are independent predictors of reoperation after complete AVSD repair. Biventricular repair of isolated AVSD with a small left ventricle can be successfully accomplished with no mortality.

Long-term follow-up after primary complete repair of common arterial trunk with homograft: a 40-year experience.

BACKGROUND: We sought to determine the long-term performance of homograft and truncal valve after complete repair of common arterial trunk. METHODS: From January 1964 to June 2008, 32 patients (median age, 14 days; range, 5 days to 2.5 years) underwent primary homograft repair of common arterial trunk. Twenty-four (75%) were neonates. The homograft used in the right ventricular outflow tract was aortic in 24 patients and pulmonary in 8 patients (mean diameter, 15.8 +/- 3.5 mm; median diameter, 16 mm [range, 8-24 mm]). The median follow-up was 24.5 years (range, 5.6 months to 43.5 years). RESULTS: There were 3 hospital deaths and 1 late death. The actuarial survival at 30 years was 83.1% +/- 6.6%. Of the 28 survivors, 25 reoperations were performed in 19 (76%) patients. The mean and median times to homograft reoperation were 11.5 +/- 7.4 and 12.1 years (range, 1.0-26.1 years), respectively. Overall freedom from homograft reoperation after 10, 20, and 30 years was 68.4% +/- 8.7%, 37.4% +/- 9.5%, and 26.7% +/- 9.3%, respectively. Twelve patients retained the original homografts at a median follow-up of 16.4 years (range, 0-30.2 years). Six underwent a truncal valve replacement with a mechanical prosthesis at a median of 10.5 years (range, 3.4-22 years) after truncus repair. Freedom from truncal valve replacement at 10 and 30 years was 93.1% +/- 4.7% and 81.8% +/- 8.9%, respectively. In the 22 surviving patients who did not undergo truncal valve replacement, the peak truncal valve gradient was 8.9 +/- 8.3 mm Hg at a median follow-up of 24.5 years (range, 5.6 months to 32.9 years). At the last follow-up, 27 (96.4%) patients had good left ventricular function, and 24 patients (85.7%) were New York Heart Association class I. CONCLUSIONS: Oversizing the homograft at the time of the initial repair can lead to a homograft lasting more than 12 years. During long-term follow-up, 20% of patients require truncal valve replacement.

Sildenafil therapy in complex pulmonary atresia with pulmonary arterial hypertension.

BACKGROUND: Complex pulmonary atresia (CPA) i.e. pulmonary atresia with ventricular septal defect and major aorto-pulmonary collaterals (MAPCAs) or Tetralogy of Fallot with MAPCAs frequently have a clinical course complicated by development of pulmonary arterial (PA) hypertension. METHODS: A cross-sectional retrospective review of patients >16 years with CPA or Tetralogy of Fallot with MAPCAs and PA hypertension treated with sildenafil was conducted. Case notes were reviewed for baseline and follow-up (after sildenafil) characteristics. RESULTS: Five patients, 4 female, median age 28 (range 18 to 47) years, were identified. All patients experienced symptomatic improvement: 2 of 4 wheelchair bound patients responded dramatically and walked 345 and 157 m respectively in 6 min following sildenafil therapy. One of the 4 with marked PA arborization abnormalities and severe ventricular dysfunction had initial symptomatic improvement. Another patient improved from walking less 100 m to climbing 2 flights of stairs. Arterial saturations improved in 2 cases from 70 and 60% to 87 and 84% respectively, whilst arterial saturations remained static in 1 case despite embolization of a classical Blalock-Taussig shunt. One patient with PA arborization/diminished PA bed was unable to tolerate sildenafil. CONCLUSIONS: Sildenafil is well tolerated and leads to symptomatic improvement and better saturations in the majority of patients with CPA with PA hypertension when used in isolation or as an adjunct to percutaneous PA angioplasty.

Sildenafil therapy for pulmonary arterial hypertension associated with atrial septal defects.

BACKGROUND: There is increasing evidence for beneficial effects of the type 5 phosphodiesterase inhibitor, sildenafil, in idiopathic pulmonary arterial hypertension (PAH). The effects of sildenafil in severe PAH associated with an atrial septal defect (ASD) have not been clearly delineated. METHODS: This extended case series reviews our experience with sildenafil treatment in three patients with severe PAH and Eisenmenger syndrome associated with an ASD. Case notes were reviewed for baseline and follow-up anatomic, clinical and haemodynamic characteristics. RESULTS: Of three patients identified, median age 44.3 years (range, 28 to 59 years), two had large secundum ASDs and the other a sinus venous defect. All patients had severe PAH and were desaturated at rest and/or during exercise. Sildenafil was started because of progressive and severe effort intolerance. All patients experienced symptomatic improvement, had higher arterial saturations (range 8-19%) and improvement in effort tolerance (88 m and 56 m improvement in 6-min walk test distance (MWTD) in two patients). Right ventricular (RV) function and Doppler derived RV systolic pressure improved in two patients. Pulmonary arterial pressures decreased in two patients who had cardiac catheterization (range 4 to 14 mm Hg). Pulmonary vascular resistance decreased from 7.58 to 3.8 Wood's units in one patient who is now awaiting surgery. Another patient developed significant pulmonary vasoreactivity (8.3 to 6.2 Wood's units with 100% oxygen) after 16 months of sildenafil therapy. CONCLUSIONS: Sildenafil not only relieves symptoms associated with severe PAH in patients with large ASDs, but also improves pulmonary arterial hemodynamics and RV function.

Hepatic changes in the failing Fontan circulation.

BACKGROUND: The failing Fontan circulation is associated with hepatic impairment. The nature of this liver injury is poorly defined. OBJECTIVE: To establish the gross and histological liver changes of patients with Fontan circulation relative to clinical, biochemical and haemodynamic findings. METHODS: Patients were retrospectively assessed for extracardiac Fontan conversion between September 2003 and June 2005, according to an established clinical protocol. Twelve patients, mean age 24.6 (range 15.8-43.4) years were identified. The mean duration since the initial Fontan procedure was 14.1 (range 6.9-26.4) years. RESULTS: Zonal enhancement of the liver (4/12) on CT was more common in patients with lower hepatic vein pressures (p = 0.007), and in those with absent cardiac cirrhosis on histological examination (p = 0.033). Gastro-oesophageal varices (4/12) were more common in patients with higher hepatic vein pressure (21 (6.3) vs 12.2 (2.2) mm Hg, p = 0.013) and associated with more advanced cirrhosis (p = 0.037). The extent of cirrhosis (7/12) was positively correlated with the hepatic vein pressure (r = 0.83, p = 0.003). A significant positive correlation was found between the Fontan duration and the degree of hepatic fibrosis (r = 0.75, p = 0.013), as well as presence of broad scars (r = 0.71, p = 0.021). Protein-losing enteropathy (5/12) occurred more frequently in patients with longer Fontan duration (11.7 (3.2) vs 17.9 (6.1) years, p = 0.038). CONCLUSIONS: Liver injury, which can be extensive in this patient group, is related to Fontan duration and hepatic vein pressures. CT scan assists non-invasive assessment. Cardiac cirrhosis with the risk of developing gastro-oesophageal varices and regenerative liver nodules, a precursor to hepatocellular carcinoma, is common in this patient group.

New technique for fenestration of the interatrial septum.

Achieving controlled flow between the systemic and pulmonary venous circulations is desirable in many complex congenital heart diseases. This includes the Fontan circulation, primary pulmonary hypertension, double inlet ventricles, or hypoplastic left heart with obstruction to the atrioventricular valve. As no specific device is available for this purpose, we developed a balloon-mounted stent technique to achieve a predetermined-sized fenestration of an atrial baffle in a patient with Fontan circulation. The details of the technique are described.

Esmolol-assisted balloon and stent angioplasty for aortic coarctation.

The objective of this study was to evaluate the effectiveness and safety of esmolol-induced negative ino- and chronotropism during stent/balloon angioplasty for aortic coarctation. Balloon angioplasty and stent placement have become widely accepted therapies for native and recurrent coarctation of the aorta (CoA). Trauma to the vessel wall and stent migration related to forward displacement of the balloon and/or stent by cardiac output, are the most common complications. Controlling stroke volume and heart rate may assist in balloon stability and accurate deployment of stents. All methods currently used to achieve this have significant limitations. We describe our experience using esmolol to control stroke volume and heart rate during balloon/stent angioplasty of CoA. We performed a retrospective review of all patients who had intravenous esmolol during percutaneous treatment of CoA. Six interventions were performed in six patients: coarctation stent angioplasty in five patients (two native coarctation) and balloon angioplasty alone in one patient. The median systolic blood pressure achieved during the procedure was 65 mmHg (range, 57-75) representing a median reduction of 40 mmHg (range, 20-80; p = 0.008) from baseline. The median heart achieved was 50 beats/min (range, 20-80), representing a median reduction of 20 beats/min (range, 15-90, p = 0.048) from baseline. Optimal stent position was obtained in all patients. Intravenous esmolol controls periprocedural hemodynamics effectively and safely during percutaneous therapy for aortic coarctation, thereby aiding accurate stent placement. Further evaluation of its use during other percutaneous left heart interventions is required.