Cetta, Frank

ObjectivesOur objective was to determine if technical changes combined with radiation safety initiatives reduced the radiation dose delivered to patients during congenital catheterization.BackgroundUse of ionizing radiation is necessary during cardiac catheterization. Minimizing radiation dose, while maintaining clinically useful image quality, is an important safety issue. In our congenital heart center intentional practice changes, including technical changes and provider awareness initiatives, were implemented to decrease radiation dose.MethodsData were retrospectively collected for all procedures involving children and adults with congenital heart disease (CHD) undergoing catheterization over 45 months. Cases were divided into three categories including: noninterventional (NI), simple intervention (SI), and complex intervention (CI). The change in dose was modeled as log of cumulative air kerma (Ka,r). The change in Ka,r was evaluated for each procedural category as well as changes occurring as a function of age and weight.ResultsConsidering all procedures (n = 1,082), Ka,r decreased by 61%. In the NI group (n = 481), Ka,r decreased by 71%. In the SI group (n = 424), Ka,r decreased by 74%. The Ka,r for the 10–17 year old group (n = 125) and those ≥18 years (n = 709) decreased 74 and 67%, respectively. The Ka,r decreased 72 and 66% for those 20–60 kg and ≥60 kg, respectively. Groups not showing significant change in Ka,r included CI, age ≤9 years, and weight ≤20 kg.ConclusionsThrough technical changes and provider awareness initiatives, our institution dramatically reduced the radiation dose in the majority of pediatric and adult CHD patients undergoing cardiac catheterization. © 2014 Wiley Periodicals, Inc.

The purpose of this case is to describe the complex perioperative management of a 30-year-old woman with congenital heart disease and multiple resternotomies presenting with pulmonary homograft dysfunction and evaluation for percutaneous pulmonary valve replacement. Transvenous, transcatheter Melody valve placement caused left main coronary artery occlusion and cardiogenic shock. An Impella ventricular assist device (VAD) provided rescue therapy during operating room transport for valve removal and pulmonary homograft replacement. ECMO support was required following surgery. Several days later during an attempted ECMO wean, her hemodynamics deteriorated abruptly. Transesophageal and epicardial echocardiography identified pulmonary graft obstruction, requiring homograft revision due to large thrombosis. This case illustrates a role for Impella VAD as bridge to definitive procedure after left coronary occlusion and describes management of complex perioperative ECMO support challenges.

ObjectivesWe set to measure the interatrial pressure gradient during simulated obstructive sleep apnea (OSA).BackgroundOSA occurs when a sleeping patient attempts to inhale against an obstructed airway. How this event affects the interatrial pressure gradient has not been defined. We hypothesized that simulated OSA in a conscious subject (Mueller maneuver [MM], inspiration against obstruction) would promote increased right‐to‐left pressure gradient, and then the substrate for right‐to‐left atrial shunting.MethodsSelected patients underwent simultaneous measurement of airway and atrial pressures (both left and right atrium [LA, RA]) using high‐fidelity micromanometry at rest, during MM, and during VM, during right heart catheterization.ResultsTen patients (age 55 ± 11 years, two women) were successfully studied. During the onset of MM, RA pressure transiently but consistently exceeded LA pressure in response to the steep decline in intrathoracic pressure (maximum RA‐LA pressure gradient increased from 0.1 ± 1.4 mm Hg at baseline to 7.0 ± 4.3 mm Hg during MM, P < 0.001). The maximum right‐to‐left atrial pressure gradient during Mueller maneuver was higher than that achieved during the Valsalva maneuver release (P < 0.007).ConclusionsThe onset of MM increased right‐to‐left pressure gradient across the atrial septum, likely as a result of greater blood return to the RA from extrathoracic veins. The RA‐LA pressure gradient achieved during MM was greater than that observed during VM. These findings delineate the hemodynamic substrate for right to left shunting during OSA. © 2014 Wiley Periodicals, Inc.

The acromelic dysplasias comprise short stature, hands and feet, and stiff joints. Three disorders are ascribed to this group, namely Weill–Marchesani syndrome, geleophysic dysplasia, and acromicric dysplasia, although similar in phenotype, can be distinguished clinically. Weill–Marchesani syndrome, on the basis of microspherophakia and ectopia lentis; geleophysic dysplasia by progressive cardiac valvular thickening, tracheal stenosis, and/or bronchopulmonary insufficiency, often leading to early death. Microspherophakia has not been reported previously in geleophysic dysplasia. Mutations in FBN1, ADAMTS10, or ADAMTS17 cause Weill–Marchesani syndrome by disrupting the microfibrillar environment, while geleophysic dysplasia is associated with enhanced TGF‐β signaling mediated through mutations in FBN1 or ADAMTSL2. We studied a 35‐year‐old woman with geleophysic dysplasia, with short stature, small hands and feet, limitation of joint mobility, mild skin thickening, cardiac valvular disease, restrictive pulmonary disease, and microspherophakia. Sequencing of ADAMTSL2 demonstrated two changes: IVS8‐2A>G consistent with a disease‐causing mutation, and IVS14‐7G>A with potential to generate a new splice acceptor site and result in aberrant mRNA processing. The unaffected mother carries only the IVS8‐2A>G transition providing evidence that the two changes are in trans‐configuration in our patient. © 2013 Wiley Periodicals, Inc.

ObjectivesTo compare procedural outcomes of transcatheter balloon pulmonary valvuloplasty (BPV) in adults to a concurrent pediatric cohort and to identify whether BPV leads to improvement in symptoms among adults.BackgroundBPV has long been utilized to relieve right ventricular outflow tract (RVOT) obstruction in neonates and young children. More recently, BPV has been performed in adults, but outcome data are limited in this population.MethodsWe reviewed all cases of BPV performed at Mayo Clinic's Congenital Cardiac Cath Lab from December 1993 to July 2012. Patients were excluded if they had additional congenital heart defects, other than simple left‐to‐right shunt lesions.Results89 children and 40 adults underwent 132 BPV procedures. Among the adults, 78% were symptomatic at the time of BPV. The most common symptoms were dyspnea and fatigue. RVOT systolic gradient was 42 ± 19 mmHg (children) and 54 ± 26 mmHg (adults) prior to BPV (P = 0.002). Adults experienced a greater reduction in gradient following BPV (32 ± 21 mmHg vs. 23 ± 19 mmHg; P = 0.007). No patients exhibited “suicide right ventricle” physiology after BPV. Among adults, mean follow‐up was 1.9 years (30 days–11 years). Symptoms improved in 20 of the 24 previously symptomatic adults for whom follow‐up data were available. Mean NYHA score improved from 2.2 to 1.4 following BPV. At last follow‐up, 52% of adults were free of any symptoms, a significant improvement from pre‐BPV (P = 0.0003). Only one patient described worsening of symptoms after BPV.ConclusionsBPV is an effective procedure for symptomatic adults with pulmonary valve stenosis. In addition to immediate relief of RVOT gradient, adult patients may experience symptomatic improvement following BPV.© 2013 Wiley Periodicals, Inc.

BackgroundIn recent years, percutaneous device closure has become commonplace for treatment of atrial septal defect (ASD) and patent foramen ovale (PFO). However, the effect of device closure on aortic valve regurgitation (AR) has been questioned.ObjectiveThe purpose of this study was to define changes in AR using echocardiography, before and after device closure of ASD or PFO.MethodsWe retrospectively reviewed all patients who had percutaneous device closure of ASD or PFO at our institution between 2005 and 2009. We compared the preprocedure echocardiogram to the most recent follow‐up study. All investigators were blinded to referral diagnosis, patient demographics, and size and type of the device placed. Aortic regurgitation was graded as none/trivial, mild, moderate, or severe.ResultsOverall, 322 patients qualified for inclusion; 204 with PFO and 118 with ASD. Mean age at time of closure was 48 ± 20 years (range 3–85 years). Mean duration of follow‐up was 1.2 years (range 2 months to 5 years). Only two patients (0.6%) demonstrated any appreciable increase in AR severity. However, both of these patients manifested a clinically insignificant change from a trivial to mild grade of aortic regurgitation.ConclusionsPercutaneous device closure of PFO or ASD does not increase the incidence or severity of AR over medium‐term follow‐up. © 2012 Wiley Periodicals, Inc.

Background —The objectives of our study were to determine the frequency of supraventricular tachyarrhythmias (SVTAs) among modifications of the Fontan operation and identify risk factors for developing SVTA. Methods and Results —The population consisted of all patients who had any modification of the Fontan operation at the Mayo Clinic between 1985 and 1993. Clinically significant SVTAs were those requiring initiation or change of antiarrhythmic treatment, and they were divided into early SVTAs (<30 days after the operation) and late SVTAs (≥30 days after the operation). Clinical histories were reviewed, and health status questionnaires were sent. Four hundred ninety-nine patients had various modifications of the Fontan operation. Frequency of early SVTA was 15%. Risk factors identified by multivariate analysis for early SVTA were AV valve regurgitation, abnormal AV valve, and preoperative SVTA. Frequency of late SVTA was 6% by 1 year, 12% by 3 years, and 17% by 5 years. Risk factors for late SVTA were age at operation (<3 or ≥10 years) and systemic AV valve replacement. By univariate and multivariate analysis, the type of Fontan operation was not a significant risk factor for late SVTA when all 6 modifications were considered. However, when we analyzed the frequency of late SVTA for the 2 recently used modifications, we found a lower frequency of late SVTA in patients with atriopulmonary connection with lateral tunnel compared with those with total cavopulmonary connection. Conclusions —Postoperative SVTA continues to be a significant problem. Risk factors for SVTA are AV valve regurgitation, abnormal AV valve, preoperative SVTA, and age at operation. Frequency of SVTA does not appear to be related to type of Fontan procedure except for slightly lower frequency in patients with atriopulmonary connection with lateral tunnel compared with those with total cavopulmonary connection.

We present a newborn patient who had surgical correction of supracardiac total anomalous pulmonary venous connection repair with an unusual cause of residual postoperative pulmonary venous obstruction secondary to compression from the transesophageal echocardiography (TEE) probe.