Does the right ventricle size influence the left ventricle size and function in children with Ebstein anomaly?

PURPOSE: Patients with Ebstein anomaly (EA) have a variety of clinical manifestation. The assessment of structural and geometric characteristics of the heart is important for optimal management. METHODS: We retrospectively analyzed echocardiography database from 2009 to 2020. We evaluate patients in two groups: patients with EA were in Group 1 and children without cardiovascular pathology were in Group 2. All children in both groups underwent echocardiography according to American Society of Echocardiography recommendations. The shape of the heart chambers and their function were studied in both groups. RESULTS: There were 153 in Group 1 and 2000 children without cardiovascular disease in Group 2. It was shown that in children with EA, the shape of the ventricle became less spherical, which was accompanied by a decrease in myocardial mass, and the ejection fraction was reduced 34% of patients. The functional volume (non-atrialized part) of the right ventricle in patients with EA was reduced, and its contractility was preserved in 62% of cases. Preservation of the contractile properties of the right ventricle in most cases was associated with higher systolic pressure in its cavity. CONCLUSION: TAPSE, TESV, and the velocity of the annulus fibrous ring movement according to tissue dopplerography in patients with EA do not allow us to assess the contractility of the right ventricle. The myocardial performance index (MPI) characterizes a decrease in the functional volume of the right ventricle.

Surgery for aortic recoarctation in children less than 10 years old: A single-center experience in Siberia, Russia.

BACKGROUND: Persistence or recurrence of stenosis is a complication of initial coarctation repair. This study aims to report short-term outcomes of surgical management of recurrent coarctation and initial repair analysis. METHODS: We retrospectively reviewed our experience with 51 patients undergoing recoarctation surgical repair between 2008 and 2019 using antegrade cerebral perfusion (ACP) technique. RESULTS: Surgical correction included prosthetic patch aortoplasty in 23 (45%), resection with wide end-to-end anastomosis in 15 (29%), and a tube interposition graft in 13 (25%) patients. The median age at initial correction and reintervention was 12 months and 9 years. The median interval from primary repair to reintervention was 60 months. Initial repair analysis revealed 33% of patients had initial correction in the neonatal period, 72.5% of patients were done via a left thoracotomy approach and 63% of patients had end-to-end anastomosis at initial surgery. CONCLUSION: Our study demonstrates that surgical repair of recurrent coarctation of the aorta using ACP technique can be performed safely and with excellent results.

Surgical management of a giant congenital left ventricular aneurysm in a 2-month-old infant.

A congenital left ventricular aneurysm is very rare. Clinical presentation varies from absence of symptoms to ventricular arrhythmias, heart failure or even sudden death. The optimal management is controversial and the surgical technique is not defined. A left ventricular aneurysm was diagnosed on prenatal echocardiography at 33 weeks gestation. After birth, initial transthoracic echocardiography confirmed the diagnosis. Two months later, the infant was taken to surgery for aneurysm repair using the Dor procedure with cardiopulmonary bypass. This technique eliminates the need for external prosthetic materials and produces a more physiologic left ventricular geometry. Transthoracic echocardiography performed at 6-month follow-up showed an ejection fraction of 66%.

Surgical repair of discontinuous right pulmonary artery utilising an autologous main pulmonary artery flap: a case report and literature review.

This is a case of an infant with unilateral discontinuous right pulmonary artery. Cardiac catheterisation with pulmonary wedge injection diagnosed the anomaly and aided in surgical planning. The patient underwent semi-autologous surgical repair utilising an autologous main pulmonary artery flap. One month following discharge, he underwent successful balloon dilation of the residual stenosis and was discharged the same day.

Loss of Ventricular Function After Bidirectional Cavopulmonary Connection: Who Is at Risk?

Decline of single ventricle systolic function after bidirectional cavopulmonary connection (BDCPC) is thought to be a transient phenomenon. We analyzed ventricular function after BDCPC according to ventricular morphology and correlated this evolution to long-term prognosis. A review from Mayo Clinic databases was performed. Visually estimated ejection fraction (EF) was reported from pre-BDCPC to pre-Fontan procedure. The last cardiovascular update was collected to assess long-term prognosis. A freedom from major cardiac event survival curve and a risk factor analysis were performed. 92 patients were included; 52 had left ventricle (LV) morphology and 40 had right ventricle (RV) morphology (28/40 had hypoplastic left heart syndrome (HLHS)). There were no significant differences in groups regarding BDCPC procedure or immediate post-operative outcome. EF showed a significant and relevant decrease from baseline to discharge in the HLHS group: 59 ± 4% to 49 ± 7% or - 9% (p < 0.01) vs. 58 ± 3% to 54 ± 6% or - 4% in the non-HLHS RV group (p = 0.04) and 61 ± 4% to 60 ± 4% or - 1% in the LV group (p = 0.14). Long-term recovery was the least in the HLHS group: EF prior to Fontan 54 ± 2% vs. 56 ± 6% and 60 ± 4%, respectively (p < 0.01). With a median follow-up of 8 years post-BDCPC, six patients had Fontan circulation failure, four died, and three had heart transplantation. EF less than 50% at hospital discharge after BDCPC was strongly correlated to these major cardiac events (HR 3.89; 95% Cl 1.04-14.52). Patients with HLHS are at great risk of ventricular dysfunction after BDCPC. This is not a transient phenomenon and contributes to worse prognosis.

The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States.

The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.

Surveillance Testing and Preventive Care After Fontan Operation: A Multi-Institutional Survey.

More children with single ventricle heart disease are surviving after Fontan surgery. This circulation has pervasive effects on multiple organ systems and has unique modes of failure. Many centers have created multidisciplinary programs to care for these patients. Our aim was to survey such programs to better understand current approaches to care. We hypothesized that significant variability in surveillance testing strategy would be present. Eleven academic institutions with established Fontan care programs performing a combined estimated 300 Fontan surgeries per year, with a total population of 1500-2000 Fontan patients, were surveyed using a REDCap survey regarding surveillance testing and basic practice philosophies. Fontan care programs were structured both as consultative services (64%) and as the primary clinical team (9%). Electrocardiograms (73%) and echocardiograms (64%) were most commonly obtained annually. Serum studies, including complete blood count (73%), complete metabolic panel (73%), and Brain-type natriuretic peptide (54%), were most commonly obtained annually. Hepatic testing consisted of liver ultrasound in most centers, obtained biennially (45%) or > every 2 years (45%). Liver biopsy was not routinely recommended (54%). Neurodevelopmental outcomes were assessed at most institutions (54%), with a median frequency of every 3-4 years. There is considerable variability in the surveillance testing regimen and management strategy after a Fontan procedure at surveyed programs. There is an urgent need for surveillance guidelines to reduce variability, define quality metrics, streamline collaborative practice, and prospective research to better understand the complex adaptations of the body to Fontan physiology.

Quality of life in young patients after cone reconstruction for Ebstein anomaly.

OBJECTIVE: To evaluate the health status and quality of life of young patients who had cone reconstruction for Ebstein anomaly. METHODS: We reviewed all patients who had cone reconstruction from 2007 to 2016 at our institution. Prospective surveys were mailed to all eligible patients. Quality of life was assessed using the PedsQL 4.0 Generic Core Scales, including four domains: physical, emotional, social, and school functioning. RESULTS: Of 116 eligible patients, 72 (62%) responded. About 96% reported their health as excellent or good, and 52% were symptom-free. Only 37% of patients were taking any medications, the most common of which was aspirin (30%). Only 19% had been hospitalised for cardiac reasons following cone reconstruction. The average self-reported quality of life was 85.3/100, whereas the average parent proxy-reported quality of life was 81.8/100. There was no difference by self or parent proxy-report in quality of life between cone reconstruction patients and healthy children; however, quality of life was significantly better compared with children with other chronic health conditions. By self-report and parent proxy-report, 15.1 and 16.7% of patients were deemed "at risk" for reduced quality of life, respectively. Socially, 63/64 (98%) patients over 5 years old were either full-time students or working full-time. CONCLUSION: Children with Ebstein anomaly following cone reconstruction have excellent quality of life comparable with healthy peers and significantly better than other children with chronic health conditions. Families of children with Ebstein anomaly can expect excellent quality of life, long-term health status, and social functioning following cone reconstruction.

Hypoplastic Left Heart Syndrome: An Overview for Primary Care Providers.

Hypoplastic left heart syndrome is one of the most complex congenital heart diseases and requires several cardiac surgeries for survival. The diagnosis is usually established prenatally or shortly after birth. Each stage of surgery poses a unique hemodynamic situation that requires deeper understanding to manage common pediatric problems such as dehydration and respiratory infections. Careful multidisciplinary involvement in the care of these complex patients is improving their outcome; however, morbidity and mortality are still substantial. In this review, we focus on the hemodynamic aspects of various surgical stages that a primary care provider should know to manage these challenging patients.

Imaging the adult with transposition of the great arteries.

PURPOSE OF REVIEW: Patients with complete and congenitally corrected transposition of the great arteries commonly survive into adulthood and present with a vast array of clinical residua. RECENT FINDINGS: Echocardiography remains the primary imaging modality in the routine assessment of the adult with transposition of the great arteries. It provides a comprehensive anatomic and hemodynamic evaluation. Limitations to echocardiography include evaluation of the following: the systemic right ventricle, baffle patency following atrial switch procedure, coronary arteries following arterial switch procedure or Nikadoh, and multilevel right ventricular outflow tract obstruction. SUMMARY: Each form of palliation for transposition of the great arteries results in unique long-term sequelae that affect outcomes. A multimodality approach to imaging is required for a complete evaluation.

Long-term follow-up after PFO device closure: Outcomes and Complications in a Single-center Experience.

OBJECTIVES: To describe medium- to long-term follow-up of patients undergoing device closure of patent foramen ovale (PFO) for stroke or transient ischemic attack (TIA). BACKGROUND: Transcatheter device closure of PFO continues to be used for secondary prevention of cryptogenic stroke or TIA, but data are lacking regarding long-term outcomes. METHODS: We reviewed the Mayo Clinic Rochester PFO database for patients who underwent device closure of PFO for secondary prevention of stroke/TIA from December 2001 to June 2012. Demographic, clinical, procedural, and follow-up data were reviewed. Recurrence rate of stroke/TIA were calculated. Data were analyzed to identify risk factors of recurrent neurologic events. RESULTS: Seven hundred and thirty patients (mean age 53 years; 40% female) were included in our study. Most patients (72%) had cryptogenic stroke as their qualifying event for PFO closure. There were no procedure-related deaths or strokes. The most commonly used device was the Amplatzer Septal Occluder (82%). Median follow-up was 6 years. A 3.9% of patients had documented residual right-to-left shunting at last echocardiogram. Recurrent stroke and/or TIA were reported in 45 patients, (6.3% of patients with follow-up data). Patients who experienced recurrent stroke/TIA were older and had a higher rate of diabetes and hypertension than those who did not. The presence of residual shunt did not correlate with risk of recurrent events. CONCLUSIONS: Device closure of PFO can be performed safely with very good long-term resolution of atrial shunting. Recurrent neurologic events after PFO closure may reflect additional comorbid risk factors unrelated to the potential for paradoxical embolism. © 2016 Wiley Periodicals, Inc.

Intravascular and hybrid intraoperative stent placement for baffle obstruction in transposition of the great arteries after atrial switch.

OBJECTIVES: To report our experience with intravascular and hybrid intra-operative stent placement for baffle obstruction in patients with complete transposition of the great arteries (TGA) after the atrial switch (Mustard/Senning) operation. BACKGROUND: Venous baffle obstruction is a challenging complication after atrial switch operation in patients with TGA. Traditional treatment options include intravascular stenting or surgery. METHODS: A retrospective analysis of Mayo Clinic's electronic medical record was completed to identify consecutive pediatric and adult patients with TGA after atrial switch who underwent baffle stent implantation from 1994 to 2015. RESULTS: Overall, 64 patients were referred for cardiac catheterization, in whom 47 (73%) were noted to have hemodynamic and angiographic evidence of baffle obstruction. A total of 20 patients mean age 33 (range: 8-46) years old underwent stent implantation of baffle stenosis at a mean of 33 (range: 7.5-45) years after initial atrial switch operation (Mustard, n = 19; Senning, n = 1). Overall, 27 baffles were stented in 20 patients via the following approaches: intravascular (17); hybrid surgical (3); staged intravascular & hybrid (2). Sites of stent placement were: superior vena cava (SVC) (13); inferior vena cava (IVC) (9); pulmonary venous baffle (5). Three patients had stent placement in the SVC and IVC baffles during the same procedure. Procedural adverse events occurred in 2/22 cases (9%) including creation of unintentional baffle leak (n = 1) and stent migration (n = 1). There was no procedure-related mortality. At follow-up (median 2, range 0.02-10 years), significantly improved NYHA class and mean Doppler baffle gradient were demonstrated (P < 0.05). Mild baffle re-stenosis (mean Doppler gradient; 2-3 mmHg) occurred in two patients who have not required re-intervention. Trivial baffle leak was noted in four patients. Baffle re-intervention was only occurred in one patient. One patient with pre-procedural Class IV symptoms died 3.5 months after stent implantation. CONCLUSIONS: This study is the largest reported experience of intravascular and intra-operative hybrid stent placement for patients with TGA after atrial switch. Transcatheter and minimally invasive hybrid intraoperative surgical stent placement are an effective strategy for relief of systemic and pulmonary venous baffle obstruction. © 2016 Wiley Periodicals, Inc.

Heart transplantation after Fontan: Results from a surgical Fontan cohort.

We performed a retrospective review of outcomes after heart transplantation during long-term follow-up of a surgical cohort of 1138 Fontan patients who were followed at the Mayo Clinic. Follow-up information was obtained from medical records and a clinical questionnaire that was mailed to patients not known to be deceased at the initiation of the study. Forty-four of 1138 Fontan patients with initial or subsequent evaluation at Mayo had cardiac transplantation between 1988 and 2014 (mean age at transplantation was 23.2 ± 12 yr, median was 19.8 yr; mean interval between Fontan and transplantation was 13.0 ± 7.7 yr, median was 13.1 yr). Two patients had combined organ transplantation (one heart-lung, one heart-liver). Twelve of the 44 (27%) patients had PLE prior to transplantation. There was no difference in post-bypass Fontan pressures or incidence of late reoperations for AVV repair/replacement between transplanted and non-transplanted patients. There were 16 (36%) deaths in the transplantation cohort; seven occurred within 30 days of transplantation. Overall one, five, 10, and 15 yr post-transplantation survival was 80%, 72%, 69%, and 55%, respectively. Although this is a challenging group of patients, intermediate-term results suggest that cardiac transplantation remains a reasonable option for patients with a failed Fontan circulation.

Transcatheter closure of postmyocardial infarction, iatrogenic, and postoperative ventricular septal defects: The Mayo Clinic experience.

OBJECTIVES: To determine event-free survival after transcatheter closure of ventricular septal defect (VSD), and to identify predictors of adverse events (AE) in post myocardial infarction VSD (post-MI VSD) subgroup. BACKGROUND: There are limited data on mid-term follow-up after transcatheter VSD closure. METHODS: Retrospective review of 27 cases of transcatheter VSD closure (post-MI = 18 and non-ischemic = 9) performed from 1999 to 2013. We defined AE as death, device embolization, hemolysis requiring blood transfusion, heart block and reintervention. RESULTS: In the post-MI VSD subgroup, mean age and follow-up was 69 ± 11 and 7.3 ± 7 years, respectively. AE occurred in 8 (44%) patients (death-3, device embolization-1, hemolysis-1, surgical VSD closure-2, reintervention-1). Event-free survival was 56% at 1 month and 5 years, and all AE occurred in the periprocedural period. Cardiogenic shock (HR: 3.21, CI: 1.82-5.41, P = 0.002), and VSD closure in acute phase (HR: 2.14, CI: 1.12-4.31, P = 0.004) were independent predictors of AE. In the non-ischemic VSD subgroup, mean age and follow-up was 49 ± 15 and 8.7 ± 8 years, respectively. AE occurred in 3 (33%) patients (late death-1, surgical VSD closure-2). For the entire cohort, freedom from death was 89% and 85% at 1 month and 5 years, and event-free survival was 70% and 61% at 1 month and 5 years. CONCLUSIONS: Transcatheter closure of post-MI VSD carries a moderate risk of periprocedural complications but low event rates afterwards. By comparison, device closure of non-ischemic VSD has lower periprocedural morbidity but some patients continued to experience AE during follow-up.

Radiation reduction in pediatric and adult congenital patients during cardiac catheterization.

OBJECTIVES: Our objective was to determine if technical changes combined with radiation safety initiatives reduced the radiation dose delivered to patients during congenital catheterization. BACKGROUND: Use 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. METHODS: Data 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. RESULTS: Considering 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. CONCLUSIONS: Through 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.

Interatrial pressure gradients during simulated obstructive sleep apnea: a catheter-based study.

OBJECTIVES: We set to measure the interatrial pressure gradient during simulated obstructive sleep apnea (OSA). BACKGROUND: OSA 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. METHODS: Selected 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. RESULTS: Ten 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). CONCLUSIONS: The 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.

Similarity of geleophysic dysplasia and Weill-Marchesani syndrome.

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-ß 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.