Implementation of the International Consortium of Health Outcomes Measurement CHD standard set in patients undergoing pulmonary valve replacement.

BACKGROUND: Despite the burden of CHD, a high cost and utilization condition, an implementation of long-term outcome measures is lacking. The objective of this study is to pilot the implementation of the International Consortium of Health Outcomes Measurement CHD standard set in patients undergoing pulmonary valve replacement, a procedure performed in mostly well patients with diverse CHD. METHODS: Patients ≥ 8 years old undergoing catheterization-based pulmonary valve replacement were approached via various approaches for patient-reported outcomes, with a follow-up assessment at 3 months post-procedure. Implementation strategy analysis was performed via a hybrid type 2 design. RESULTS: Of the 74 patients undergoing pulmonary valve replacement, 32 completed initial patient-reported outcomes with variable response rates by strategy (email and in-person explanation 100%, email only 54%, and email followed by text/call 64%). Ages ranged 8-67 years (mean 30). Pre-procedurally, 34% had symptomatic arrhythmias, which improved post-procedure. For those in school, 43% missed ≥ 6 days per year, and over half had work absenteeism. Financial concerns were reported in 34%. Patients reported high satisfaction with life (50% [n = 16]) and health-related quality of life (90% [n = 26]). Depression symptoms were reported in 84% (n = 27) and anxiety in 62.5% (n = 18), with tendency towards improvement post-procedurally. CONCLUSION: Pilot implementation of the International Consortium of Health Outcomes Measurement CHD standard set in pulmonary valve replacement patients reveals a significant burden of disease not previously reported. Barriers to the implementation include a sustainable, automated system for patient-reported outcome collection and infrastructure to assess in real time. This provides an example of implementing cardiac outcomes set in clinical practice.

Enhanced Safety and Efficiency of Ambulatory Cardiology Admissions: A Quality Improvement Initiative.

Background: Pediatric cardiac patients have experienced evolving illnesses progressing to instability while awaiting inpatient admission from ambulatory settings. Admission delays and communication breakdowns increase the risk for tenuous patients. This quality improvement initiative aimed to improve safety and efficiency for patients admitted from an ambulatory Clinic to the Acute Cardiac Care Unit (ACCU) using standardized communication and admission processes within one year. Methods: An admission process map, in-clinic nurse monitoring, and communication pathways were developed and implemented. A standardized team handoff occurred via virtual huddle using illness severity, patient summary, action list, situational awareness, and synthesis. Escalation of care events and timeliness were compared pre- and postimplementation. Results: There was a reduction of transfers to the intensive care unit within 24 hours of ACCU admission from 9.2% to 3.8% (P = 0.26), intensive care unit evaluations (without transfer) from 5.6% to 0% (P = 0.06), and arrests from 3.7% to 0% (P = 0.16). After the pilot, clinic nurses monitored 100% of at-risk patients. Overall mean time from admission decision to virtual huddle decreased from 81 to 61 minutes and mean time to admission from 144 to 115 minutes, with 41% (n = 33) arriving ≤ 60 minutes (goal). The COVID-19 pandemic negatively affected admission timeliness while safety metrics remained optimized. Conclusions: Implementing a standardized admission process between the Clinic and ACCU enhanced safety by reducing admission wait time and escalation of care post-admission. Sustainable, reliable handoff processes, in-clinic monitoring, and standardized admission processes were established. The pandemic hindered admission efficiency without compromising safety.

Social Determinants of Health Including Child Opportunity Index Leading to Gaps in Care for Patients With Significant Congenital Heart Disease.

BACKGROUND: Gaps in care (GIC) are common for patients with congenital heart disease (CHD) and can lead to worsening clinical status, unplanned hospitalization, and mortality. Understanding of how social determinants of health (SDOH) contribute to GIC in CHD is incomplete. We hypothesize that SDOH, including Child Opportunity Index (COI), are associated with GIC in patients with significant CHD. METHODS AND RESULTS: A total of 8554 patients followed at a regional specialty pediatric hospital with moderate to severe CHD seen in cardiology clinic between January 2013 and December 2015 were retrospectively reviewed. SDOH factors including race, ethnicity, language, and COI calculated based on home address and zip code were analyzed. GIC of >3.25 years were identified in 32% (2709) of patients. GIC were associated with ages 14 to 29 years (P<0.001), Black race or Hispanic ethnicity (P<0.001), living ≥150 miles from the hospital (P=0.017), public health insurance (P<0.001), a maternal education level of high school or less (P<0.001), and a low COI (P<0.001). Multivariable analysis showed that GIC were associated with age ≥14 years, Black race or Hispanic ethnicity, documenting <3 caregivers as contacts, mother's education level being high school or less, a very low/low COI, and insurance status (C statistic 0.66). CONCLUSIONS: One-third of patients followed in a regional referral center with significant CHD experienced a substantial GIC (>3.25 years). Several SDOH, including a low COI, were associated with GIC. Hospitals should adopt formal GIC improvement programs focusing on SDOH to improve continuity of care and ultimately overall outcomes for patients with CHD.

Heart Failure Following Sinus Venosus Defect Closure in a Patient With Vein of Galen Malformation.

This case concerns an 18-month-old with masked congestive heart failure (CHF) from an unrepaired vein of Galen malformation and superior sinus venosus defect who progressed to severe, refractory CHF following superior sinus venosus defect repair. Partial transvenous coil embolization of a very-high-risk vein of Galen malformation resolved CHF symptoms. (Level of Difficulty: Advanced.).

Feasibility and Caregiver Receptiveness to Anxiety Screening in Pediatric Cardiology Clinic: A Pilot Study.

Children with chronic medical conditions, including heart disease, have increased susceptibility to behavioral health concerns. We sought to evaluate the feasibility and parental opinion of anxiety screening in pediatric cardiology clinic. The PROMIS Pediatric Anxiety v2.0 Short Form 8a (PA2-S8) questionnaire was administered to 48 patients presenting to pediatric cardiology clinic for follow-up care. Parents/caregivers were asked their opinion on anxiety screening in cardiology clinic. The survey was completed by 47 out of 48 participants (median age 13, range 9-17). Fourteen (30%) participants had scores suggestive of increased anxiety symptomatology. No trends were identified between PA2-S8 score and age at diagnosis (P = .13), age at survey administration (P = .28), number of lifetime procedures (P = .89), number of noncardiac specialists (P = .13), or underlying cardiac diagnoses (P = .55). Most families (76%) were in favor of the screening effort. This study suggests that anxiety screening in cardiology clinic is both feasible and well-received by families.

Mycobacterium chimaera Outbreak Management and Outcomes at a Large Pediatric Cardiac Surgery Center.

BACKGROUND: In 2012, a global outbreak of invasive Mycobacterium chimaera (M. chimaera) infection was identified in patients after cardiopulmonary bypass surgery. Investigations revealed the source to be heater-cooler unit (HCU) exhaust, with point-source contamination discovered at the LivaNova HCU manufacturing plant (London, UK). We report our experience with affected HCUs at a high-volume pediatric cardiac surgery center in the United States. METHODS: A multidisciplinary task force was established for outbreak management, including removing contaminated HCUs from service. Patients identified as exposed to affected HCUs were systematically contacted. A call center was created for patient/family inquiries, and symptomatic patients were assessed using an institutional triage protocol, including laboratory/culture data and infectious diseases consultation. RESULTS: Cardiopulmonary bypass surgeries were performed in 4276 patients (median age: 2.1 years; range: 0-48.4 years) between October 2010 and October 2016. Call center volume was highest in the first 6 weeks after patient notification, totaling 307 calls and yielding 70 clinical patient assessments. Presenting symptoms included fatigue (60%), fever (49%), night sweats (46%), myalgias (34%), and weight loss (24%). Among the 70 assessed patients, echocardiogram (n = 30), cardiac computed tomography (n = 2), cardiac magnetic resonance imaging (n = 1), and pulmonary computed tomography (n = 1) did not reveal abnormalities suggestive of active infection. Infectious diseases consultation occurred in 23 (33%) patients. Acid-fast bacilli blood cultures were obtained in 30 patients; all were negative. CONCLUSIONS: Through a highly coordinated outreach effort, no patients have been found to have M. chimaera infection in the 6 years after exposure to contaminated HCUs. Ongoing vigilance for cases that may yet manifest is needed.

Association of Myocarditis With BNT162b2 Messenger RNA COVID-19 Vaccine in a Case Series of Children.

Importance: The BNT162b2 (Pfizer-BioNTech) messenger RNA COVID-19 vaccine was authorized on May 10, 2021, for emergency use in children aged 12 years and older. Initial reports showed that the vaccine was well tolerated without serious adverse events; however, cases of myocarditis have been reported since approval. Objective: To review results of comprehensive cardiac imaging in children with myocarditis after COVID-19 vaccine. Design, Setting, and Participants: This study was a case series of children younger than 19 years hospitalized with myocarditis within 30 days of BNT162b2 messenger RNA COVID-19 vaccine. The setting was a single-center pediatric referral facility, and admissions occurred between May 1 and July 15, 2021. Main Outcomes and Measures: All patients underwent cardiac evaluation including an electrocardiogram, echocardiogram, and cardiac magnetic resonance imaging. Results: Fifteen patients (14 male patients [93%]; median age, 15 years [range, 12-18 years]) were hospitalized for management of myocarditis after receiving the BNT162b2 (Pfizer) vaccine. Symptoms started 1 to 6 days after receipt of the vaccine and included chest pain in 15 patients (100%), fever in 10 patients (67%), myalgia in 8 patients (53%), and headache in 6 patients (40%). Troponin levels were elevated in all patients at admission (median, 0.25 ng/mL [range, 0.08-3.15 ng/mL]) and peaked 0.1 to 2.3 days after admission. By echocardiographic examination, decreased left ventricular (LV) ejection fraction (EF) was present in 3 patients (20%), and abnormal global longitudinal or circumferential strain was present in 5 patients (33%). No patient had a pericardial effusion. Cardiac magnetic resonance imaging findings were consistent with myocarditis in 13 patients (87%) including late gadolinium enhancement in 12 patients (80%), regional hyperintensity on T2-weighted imaging in 2 patients (13%), elevated extracellular volume fraction in 3 patients (20%), and elevated LV global native T1 in 2 patients (20%). No patient required intensive care unit admission, and median hospital length of stay was 2 days (range 1-5). At follow-up 1 to 13 days after hospital discharge, 11 patients (73%) had resolution of symptoms. One patient (7%) had persistent borderline low LV systolic function on echocardiogram (EF 54%). Troponin levels remained mildly elevated in 3 patients (20%). One patient (7%) had nonsustained ventricular tachycardia on ambulatory monitor. Conclusions and Relevance: In this small case series study, myocarditis was diagnosed in children after COVID-19 vaccination, most commonly in boys after the second dose. In this case series, in short-term follow-up, patients were mildly affected. The long-term risks associated with postvaccination myocarditis remain unknown. Larger studies with longer follow-up are needed to inform recommendations for COVID-19 vaccination in this population.

Experience participating in the American College of Cardiology Quality NetworkTM: paediatric and adult congenital cardiology collaborative quality improvement.

BACKGROUND: The American College of Cardiology Quality Network enables national benchmarking and collaborative quality improvement through vetted metrics. We describe here our initial experience with the Quality Network. METHODS: Quarterly data for metrics pertaining to chest pain, Kawasaki disease, tetralogy of Fallot, elevated body mass index, and others were shared with the collaboratives for benchmarking. National improvement efforts focussed on counselling for elevated body mass index and 22q11.2 testing in tetralogy of Fallot. Improvement strategies included developing multi-disciplinary workgroups, educational materials, and electronic health record advances. RESULTS: Chest pain metric performance was high compared with national means: obtaining family history (90-100% versus 51-77%), electrocardiogram (100% versus 89-99%), and echocardiogram for exertional complaints (95-100% versus 74-96%). Kawasaki metric performance was high, including obtaining coronary measurements (100% versus 85-97%), prescribing aspirin (100% versus 86-99%), follow-up with imaging (100% versus 85-98%), and documenting no activity restriction without coronary aneurysms (83-100% versus 64-93%). Counselling for elevated body mass index was variable (25-75% versus 31-50%) throughout quality improvement efforts. Testing for 22q11.2 deletion in tetralogy of Fallot patients was consistently above the national mean (60-85% versus 54-68%) with improved genetics data capture. CONCLUSION: The Quality Network promotes meaningful benchmarking and collaborative quality improvement. Our high performance for chest pain and Kawasaki metrics is likely related to previous improvement efforts in chest pain management and a dedicated Kawasaki team. Uptake of counselling for elevated body mass index is variable; stronger engagement among numerous providers is needed. Recommendations for 22q11.2 testing in tetralogy of Fallot were widely recognised and implemented.

Resource reduction in pediatric chest pain: Standardized clinical assessment and management plan.

OBJECTIVE: Using a Standardized Clinical Assessment and Management Plan (SCAMP) for pediatric patients presenting to clinic with chest pain, we evaluated the cost impact associated with implementation of the care algorithm. Prior to introduction of the SCAMP, we analyzed charges for 406 patients with chest pain, seen in 2009, and predicted 21% reduction of overall charges had the SCAMP methodology been used. The SCAMP recommended an echocardiogram for history, examination, or ECG findings suggestive of a cardiac etiology for chest pain. DESIGN: Resource utilization was reviewed for 1517 patients (7-21 years) enrolled in the SCAMP from July 2010 to April 2014. RESULTS: Compared to the 2009 historic cohort, patients evaluated by the SCAMP had higher rates of exertional chest pain (45% vs 37%) and positive family history (5% vs 1%). The SCAMP cohort had fewer abnormal physical examination findings (1% vs 6%) and abnormal electrocardiograms (3% vs 5%). Echocardiogram use increased in the SCAMP cohort compared to the 2009 historic cohort (45% vs 41%), whereas all other ancillary testing was reduced: exercise stress testing (4% SCAMP vs 28% historic), Holter (4% vs 7%), event monitors (3% vs 10%), and MRI (1% vs 2%). Total charges were reduced by 22% ($822 625) by use of the Chest Pain SCAMP, despite a higher percentage of patients for whom echocardiogram was recommended compared to the historic cohort. CONCLUSIONS: The Chest Pain SCAMP effectively streamlines cardiac testing and reduces resource utilization. Further reductions can be made by algorithm refinement regarding echocardiograms for exertional symptoms.

Needles in Hay II: Detecting Cardiac Pathology by the Pediatric Chest Pain Standardized Clinical Assessment and Management Plan.

OBJECTIVES: To determine if patients evaluated using the pediatric chest pain standardized clinical assessment and management plan (SCAMP) in cardiology clinic were later diagnosed with unrecognized cardiac pathology, and to determine if other patients with cardiac pathology not enrolled in the SCAMP would have been identified using the algorithm. STUDY DESIGN: Patients 7-21 years of age, newly diagnosed with hypertrophic or dilated cardiomyopathy, coronary anomalies, pulmonary embolus, pulmonary hypertension, pericarditis, or myocarditis were identified from the Boston Children's Hospital (BCH) cardiac database between July 1, 2010 and December 31, 2012. Patients were cross-referenced to the SCAMP database or retrospectively assessed with the SCAMP algorithm. RESULTS: Among 98 patients with cardiac pathology, 34 (35%) reported chest pain, of whom 10 were diagnosed as outpatients. None of these patients were enrolled in the SCAMP because of alternate chief complaints (n = 4) or referral to BCH for management of the new diagnosis (n = 6). Each of these patients would have had an echocardiogram recommended by retrospective application of the SCAMP algorithm. Two other patients with cardiac pathology were among the 1124 patients assessed by the SCAMP. One patient initially diagnosed with noncardiac chest pain presented 18 months later and was diagnosed with myocarditis as an inpatient. One patient seen initially in the emergency department was subsequently diagnosed with pericarditis as an outpatient. CONCLUSIONS: Patients assessed by the chest pain SCAMP at BCH were not later diagnosed with cardiac pathology that was missed at the initial encounter. Nonenrolled outpatients with cardiac pathology and chest pain would have been successfully identified with the SCAMP algorithm.

Accelerated degeneration of a bovine pericardial bioprosthetic aortic valve in children and young adults.

BACKGROUND: Experience with aortic valve replacement (AVR) with current-generation pericardial bioprostheses in young patients is limited. The death of a child with accelerated bioprosthetic aortic stenosis prompted enhanced surveillance of all such patients at our institution. METHODS AND RESULTS: We reviewed records of 27 patients who had undergone AVR (median follow-up, 13.7 months) with a bovine pericardial bioprosthesis at ≤30 years of age. In the Mitroflow LXA valve group (n=15), freedom from valve failure was 100% at 1 year, 53% (95% confidence interval, 12-82) at 2 years, and 18% (95% confidence interval, 1-53) at 3 years. No Magna/Magna Ease valves (n=12) failed by 3 years. Among valve failure patients, median age at AVR was 12 years (range, 10-21 years). Life-threatening prosthetic aortic stenosis was detected at a median of 6 months after prior echocardiograms showing mild or less gradients. Patients with Mitroflow LXA compared with Magna/Magna Ease valves were smaller (median body surface area, 1.42 versus 1.93 m(2); P=0.002) and younger (median age, 13.0 versus 20.9 years; P=0.02) at AVR. Pathology demonstrated diffuse intrinsic leaflet calcification, not associated with inflammation or infection, and virtually immobile leaflets in closed position. CONCLUSIONS: Young patients undergoing AVR with Mitroflow LXA pericardial valves are at high risk for rapid progression from mild or less to severe aortic stenosis over months, highlighting their need for heightened echocardiographic surveillance and suggesting that this aortic bioprosthesis should not be implanted in the young. Current data are insufficient to assess the safety of AVR with other pericardial bioprostheses in children and the youngest adults.

Resource Utilization Reduction for Evaluation of Chest Pain in Pediatrics Using a Novel Standardized Clinical Assessment and Management Plan (SCAMP).

BACKGROUND: Chest pain is a common reason for referral to pediatric cardiologists. Although pediatric chest pain is rarely attributable to serious cardiac pathology, extensive and costly evaluation is often performed. We have implemented a standardized approach to pediatric chest pain in our pediatric cardiology clinics as part of a broader quality improvement initiative termed Standardized Clinical Assessment and Management Plans (SCAMPs). In this study, we evaluate the impact of a SCAMP for chest pain on practice variation and resource utilization. METHODS AND RESULTS: We compared demographic variables, clinical characteristics, and cardiac testing in a historical cohort (n=406) of patients presenting to our outpatient division for initial evaluation of chest pain in the most recent pre-SCAMP calendar year (2009) to patients enrolled in the chest pain SCAMP (n=364). Demographic variables including age at presentation, sex, and clinical characteristics were similar between groups. Adherence to the SCAMP algorithm for echocardiography was 84%. Practice variation decreased significantly after implementation of the SCAMP (P<0.001). The number of exercise stress tests obtained was significantly lower in the SCAMP-enrolled patients compared with the historic cohort (∼3% of patients versus 29%, respectively; P<0.001). Similarly, there was a 66% decrease in utilization of Holter monitors and 75% decrease in the use of long-term event monitors after implementation of the chest pain SCAMP (P=0.003 and P<0.001, respectively). The number of echocardiograms obtained was similar between groups. CONCLUSIONS: Implementation of a SCAMP for evaluation of pediatric chest pain has lead to a decrease in practice variation and resource utilization. (J Am Heart Assoc. 2012;1:jah3-e000349 doi: 10.1161/JAHA.111.000349.).

Reproducibility of echocardiographic diagnosis of left ventricular noncompaction.

BACKGROUND: Left ventricular noncompaction (LVNC) cardiomyopathy is variably defined by numerous trabeculations, deep intertrabecular recesses, and noncompacted-to-compacted (NC/C) ratio >2. Limited studies exist on the reproducibility of diagnosing LVNC. METHODS: Clinical records of patients diagnosed with LVNC by echocardiography were reviewed. Blinded review of the index echocardiogram for all patients and a 1:1 match without LVNC was performed independently by two observers, measuring the number of trabeculations and the NC/C ratio. RESULTS: A total of 104 patients with LVNC were included in the study, 52 with no congenital heart disease (NCongHD) and 52 with congenital heart disease (CongHD). The duration of follow-up was 7.2 years (range, 0.5-23.1 years) for NCongHD and 8.2 years (range, 0-33.3 years) for CongHD. Agreement between observers in determining zero to three versus more than three trabeculations was 59% (NCongHD) and 73% (CongHD). Agreement in measuring an NC/C ratio ≤ 2 versus > 2 was 79% (NCongHD) and 74% (CongHD). Agreement with the original reader in diagnosing LVNC was 67%. There was no association between the number of trabeculations or the NC/C ratio and the likelihood of a major event. Patients with moderate or severe left ventricular dysfunction at the time of diagnosis were more likely to undergo cardiac transplantation or die compared with those with normal or mild dysfunction (NCongHD, 22% vs 0%, P = .01; CongHD, 39% vs 3%, P = .001). CONCLUSIONS: The reproducibility of making measurements to diagnose LVNC by accepted criteria is poor. Heart transplantation and death are associated with significant ventricular dysfunction and not with increased trabeculations or NC/C ratios.

Effectiveness of screening for life-threatening chest pain in children.

OBJECTIVE: We sought to determine the incidence of sudden cardiac death among patients discharged from the cardiology clinic with presumed noncardiac chest pain (CP). METHODS: The records of children >6 years of age who presented to Children's Hospital Boston between January 1, 2000, and December 31, 2009, with a complaint of CP were reviewed for demographic features, clinical characteristics, resource utilization, and presumed diagnosis. Patients were searched for in the US National Death Index and Social Security Death Index. RESULTS: Data for a total of 3700 patients with CP (median age at evaluation: 13.4 years [range: 7-22.3 years]) were reviewed. The median follow-up period was 4.4 years (range: 0.5-10.4 years), for total of 17 886 patient-years of follow-up data. CP with exertion occurred in 1222 cases (33%), including 15 with associated syncope. A cardiac cause was determined in 37 cases; the remaining 3663 patients (99%) had CP of unknown (n = 1928), musculoskeletal (n = 1345), pulmonary (n = 242), gastrointestinal (n = 108), anxiety-related (n = 34), or drug-related (n = 4) origin. Emergency department visits for CP were documented for 670 patients (18%), and 263 patients (7%) had cardiology follow-up visits related to CP. There were 3 deaths, including 2 suicides and 1 spontaneous retroperitoneal hemorrhage. CONCLUSION: CP in children is a common complaint and rarely has a cardiac cause. Review of 1 decade of cardiology visits (nearly 18 000 patient years) revealed that no patient discharged from the clinic died as a result of a cardiac condition.

Management of pediatric chest pain using a standardized assessment and management plan.

OBJECTIVES: Chest pain is a common reason for referral to pediatric cardiologists and often leads to an extensive cardiac evaluation. The objective of this study is to describe current management practices in the assessment of pediatric chest pain and to determine whether a standardized care approach could reduce unnecessary testing. PATIENTS AND METHODS: We reviewed all patients, aged 7 to 21 years, presenting to our outpatient pediatric cardiology division in 2009 for evaluation of chest pain. Demographics, clinical characteristics, patient outcomes, and resource use were analyzed. RESULTS: Testing included electrocardiography (ECG) in all 406 patients, echocardiography in 175 (43%), exercise stress testing in 114 (28%), event monitoring in 40 (10%), and Holter monitoring in 30 (7%). A total of 44 (11%) patients had a clinically significant medical or family history, an abnormal cardiac examination, and/or an abnormal ECG. Exertional chest pain was present in 150 (37%) patients. In the entire cohort, a cardiac etiology for chest pain was found in only 5 of 406 (1.2%) patients. Two patients had pericarditits, and 3 had arrhythmias. We developed an algorithm using pertinent history, physical examination, and ECG findings to suggest when additional testing is indicated. Applying the algorithm to this cohort could lead to an ∼20% reduction in echocardiogram and outpatient rhythm monitor use and elimination of exercise stress testing while still capturing all cardiac diagnoses. CONCLUSIONS: Evaluation of pediatric chest pain is often extensive and rarely yields a cardiac etiology. Practice variation and unnecessary resource use remain concerns. Targeted testing can reduce resource use and lead to more cost-effective care.

Anatomic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle.

The purposes of this study were to characterize the morphologic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle (DI-DORV), a rare congenital heart disease, and to compare these findings to those in patients with double-inlet left ventricle (DILV). The clinical records, imaging studies, and heart specimens of 22 consecutive patients with DI-DORV were reviewed. A comparison group of 44 patients with DILV was matched by age at latest follow-up. Among the 22 patients with DI-DORV, 14 had clinical data, 5 had clinical and autopsy data, and 3 had only autopsy data. Abdominal and atrial situs were normal in all, and heart position was levocardia in 91%. The morphology of the atrioventricular valves was variable, with 15 patients showing neither a typical tricuspid nor mitral valve. In the remaining 7 patients, 1 or both atrioventricular valves resembled a tricuspid or a mitral valve. Myocardial architecture was characterized by atypical muscle bundles of varying degrees of hypertrophy and orientation. Most patients had bilateral conus, and 82% had either aortic or pulmonary outflow tract obstructions. The rate of overall mortality or heart transplantation was 36%. Significant differences between DI-DORV and DILV included the type of ventricular loop, the type of conus, and a higher mortality rate in patients with DI-DORV. In conclusion, DI-DORV is a distinct type of functional single ventricle congenital heart disease with variable atrioventricular valve morphology and myocardial architecture. Accurate diagnosis using noninvasive imaging techniques is of paramount importance for optimal management.

Frequency of development of aortic cuspal prolapse and aortic regurgitation in patients with subaortic ventricular septal defect diagnosed at <1 year of age.

The natural history of aortic cuspal prolapse and aortic regurgitation (AR), studied most commonly in subpulmonic ventricular septal defect (VSD), has not been well defined in isolated, unrepaired VSD diagnosed during infancy. This study aimed to define the incidence and progression of aortic cuspal prolapse and AR in patients with subaortic VSDs diagnosed at <1 year of age who had no aortic cuspal prolapse or AR at presentation and did not require surgery within the first year of life. Patients had yearly follow-up, and data regarding clinical course, physical examination, and echocardiography were obtained. Comparisons were made between patients who developed aortic cuspal prolapse and AR and those who did not. One hundred patients, with a mean age at VSD diagnosis of 0.1 +/- 0.5 years, followed for a mean of 7.1 +/- 10.1 years, were studied. Aortic cuspal prolapse developed in 14 patients (14%) at a mean age of 7.1 +/- 6 years (range 0.4 to 18.4). AR murmurs were heard in 6 patients (6%) at a mean age of 5.1 +/- 3.1 years, all of whom had aortic cuspal prolapse and underwent VSD closure and aortic valvuloplasty. In conclusion, aortic cuspal prolapse and clinical AR are not uncommon in patients with subaortic VSDs. Long-term follow-up of patients with subaortic VSDs should include the serial evaluation of aortic valve anatomy and function.