Unicuspid Aortic Valve With Severe Aortic Stenosis and Progressive Heart Failure During Pregnancy.

A 39-year-old gravida 7 para 6 woman with unicuspid aortic valve and severe symptomatic stenosis was admitted to the hospital at 15 weeks gestation. We describe maternal cardiovascular complications and their implication on obstetric and fetal care. We also describe our multidisciplinary approach to caring for this complex patient.

Risk Factors for Perioperative Morbidity in Adults Undergoing Cardiac Surgery at Children's Hospitals.

BACKGROUND: Increasing numbers of adults with congenital heart disease (ACHD) undergo cardiac surgical procedures in children's hospitals, yet surgical outcomes data are limited. We sought to better understand the impact of preoperative risk factors on postoperative complications and cardiac intensive care unit (CICU) length of stay (LOS). METHODS: Surgical CICU admissions for patients aged 18 years and older in the Pediatric Cardiac Critical Care Consortium registry from August 2014 to January 2019 in 34 hospitals were included. Primary outcomes included prolonged LOS (defined as LOS ≥90th percentile) and major complications (cardiac arrest, extracorporeal membrane oxygenation, arrhythmia requiring intervention, stroke, renal replacement therapy, infection, and reoperation/reintervention). RESULTS: We analyzed 1764 surgical CICU admissions. Prolonged LOS was 7 days or longer. Eighteen patients (1.0%) died, of whom 9 (0.5%) died before the LOS cutoff and were excluded from analysis. Of 1755 CICU admissions, 8.8% (n = 156) had prolonged LOS, and 23.3% (n = 413) had 1 or more major complications. Several variables, including The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery 4/5 operation, 3 or more previous sternotomies, and preoperative renal dysfunction/dialysis were independent risk factors for both prolonged LOS and major complications (P < .05). Preoperative ventilation was associated with increased odds of prolonged LOS and preoperative arrhythmia with major complications. CONCLUSIONS: This analysis of postoperative ACHD care in pediatric CICUs found high complexity operations, 3 or more previous sternotomies, preoperative arrhythmias, renal dysfunction, and respiratory failure are associated with prolonged LOS and/or major complications. Future quality improvement initiatives focused on preoperative optimization and implementation of adult-specific perioperative protocols may mitigate morbidity in these patients undergoing cardiac surgical procedures at children's hospitals.

Pregnancy in Women with Adult Congenital Heart Disease.

Women with congenital heart disease are pursuing pregnancy in increasing numbers. Counseling about genetic transmission, medication management, maternal and fetal risks, and maternal longevity should be initiated well before pregnancy is considered. Although preconception medical and surgical optimization as well as coordinated multidisciplinary care throughout pregnancy decrease maternal and fetal risks, the rate of complications remains increased compared with the general population. Lesion-specific risk stratification and care throughout pregnancy further improve outcomes and decrease unnecessary interventions.

Aortopathy in Congenital Heart Disease.

Aortic dilatation is common in patients with congenital heart disease and is seen in patients with bicuspid aortic valve and those with conotruncal congenital heart defects. It is important to identify patients with bicuspid aortic valve at high risk for aortic dissection. High-risk patients include those with the aortic root phenotype and those with syndromic or familial aortopathies including Marfan syndrome, Loeys-Dietz syndrome, and Turner syndrome. Aortic dilatation is common in patients with conotruncal congenital heart defects and rarely results in aortic dissection.

Coronavirus Disease 2019 (COVID-19) Pandemic Implications in Pediatric and Adult Congenital Heart Disease.

The corona virus disease -2019 (COVID-19) is a recently described infectious disease caused by the severe acute respiratory syndrome corona virus 2 with significant cardiovascular implications. Given the increased risk for severe COVID-19 observed in adults with underlying cardiac involvement, there is concern that patients with pediatric and congenital heart disease (CHD) may likewise be at increased risk for severe infection. The cardiac manifestations of COVID-19 include myocarditis, arrhythmia and myocardial infarction. Importantly, the pandemic has stretched health care systems and many care team members are at risk for contracting and possibly transmitting the disease which may further impact the care of patients with cardiovascular disease. In this review, we describe the effects of COVID-19 in the pediatric and young adult population and review the cardiovascular involvement in COVID-19 focusing on implications for patients with congenital heart disease in particular.

Symptomatic Subclavian Steal During Pregnancy in a Woman Status Post Coarctation Repair in Infancy.

A 24-year-old woman with a history of coarctation repair by subclavian flap aortoplasty presented at 15 weeks' gestation with transient episodes of vision loss. She was diagnosed with subclavian steal syndrome and underwent left carotid artery to subclavian artery bypass at 17 weeks' gestation. She has had no recurrence of symptoms at ten months of postoperative follow-up. Despite the anatomic substrate for subclavian steal in patients with this type of surgical repair, neurologic symptoms are uncommon. It is possible that the pregnancy-induced fall in systemic vascular resistance triggered symptoms in this previously asymptomatic patient.

Echocardiographic predictors of elevated left ventricular end diastolic pressure in adolescent and adult patients with repaired tetralogy of Fallot.

Elevated left ventricular end diastolic pressure is a risk factor for ventricular arrhythmias in patients with tetralogy of Fallot. The objective of this retrospective study was to identify echocardiographic measures associated with left ventricular end diastolic pressure >12 mmHg in this population. Repaired tetralogy of Fallot patients age ≥13 years, who underwent a left heart catheterisation within 7 days of having an echocardiogram were evaluated. Univariate comparison was made in echocardiographic and clinical variables between patients with left ventricular end diastolic pressure >12 versus ≤12 mmHg. Ninety-four patients (54% male) with a median age of 24.6 years were included. Thirty-four (36%) had left ventricular end diastolic pressure >12 mmHg. Patients with left ventricular end diastolic pressure >12mmHg were older (median 32.9 versus 24.0 years, p = 0.02), more likely to have a history of an aortopulmonary shunt (62% versus 38%, p = 0.03), and have a diagnosis of hypertension (24% versus 7%, p = 0.03) compared to those with left ventricular end diastolic pressure ≤12 mmHg. There were no significant differences in mitral valve E/A ratio, annular e' velocity, or E/e' ratio between patients with left ventricular end diastolic pressure >12 versus ≤12 mmHg. Patients with left ventricular end diastolic pressure >12mmHg had larger left atrial area (mean 17.7 versus 14.0 cm2, p = 0.03) and larger left atrium anterior-posterior diameter (mean 36.0 versus 30.6 mm, p = 0.004). In conclusion, typical echocardiographic measures of left ventricular diastolic dysfunction may not be reliable in tetralogy of Fallot patients. Prospective studies with the use of novel echocardiographic measures are needed.

Transition of Care in Congenital Disease: Allaying Fears for Patients and Specialists.

Advances in the care of infants and children with congenital heart disease (CHD) have resulted in significantly improved survival of this population into adulthood. Although the majority of patients with CHD have undergone surgical intervention or transcatheter intervention, patients are not "cured" and almost all require lifelong cardiac care. In many parts of the world, robust systems of care have been developed for infants and children with CHD. Although there has been progress in the development of programs for the care of adults with CHD, the time of transition and transfer of care remains a time of high risk for lapses in care and becoming lost to follow-up. Transition is a process of education and preparation for young patients to assume their own care as they enter adulthood, while transfer is the actual event of a patient moving their care from a pediatric to an adult environment. The purpose of this review is to summarize what is known of best practices related to the transition and transfer of patients with CHD to adult services and to provide a practical approach to the sometimes daunting task of preparing patients for the transfer of care.

Usefulness of right ventricular free wall strain to predict quality of life in "repaired" tetralogy of Fallot.

After repair of tetralogy of Fallot, the left ventricular ejection fraction and the right ventricular ejection fraction are associated with clinical status and outcomes, but the relation of strain, a potentially earlier marker of dysfunction, to quality of life has not been evaluated. In 58 patients with tetralogy of Fallot (median age 29 years, interquartile range 20 to 41) who underwent cardiovascular magnetic resonance imaging and completed the Short Form 36, Version 2 (a validated quality-of-life assessment), left ventricular global circumferential strain, left ventricular global longitudinal strain, and right ventricular free wall longitudinal strain (RVLSFW) were measured from cine images using feature-tracking software. Age-adjusted z score ≤-1 for the physical component summary or subscales of physical functioning, role-physical, and general health was considered a clinically significant decrease in quality of life. Patients with RVLSFW less than the median had increased odds of decreased physical functioning (odds ratio [OR] 5.4, p = 0.01) and general health (OR 3.5, p = 0.04) subscale scores, which remained significant in patients with right ventricular ejection fractions ≥45% (physical functioning: OR 9.5, p = 0.03; general health: OR 5.9, p = 0.04). Left ventricular global circumferential strain and left ventricular global longitudinal strain did not predict decreased quality of life in this population. Intraobserver and interobserver variability was acceptable for left ventricular global circumferential strain (coefficients of variation 9.5% and 10.0%, respectively) but lower for left ventricular global longitudinal strain (coefficients of variation 17.2% and 16.8%, respectively) and poor for RVLSFW (coefficients of variation 19.9% and 28.8%, respectively). In conclusion, RVLSFW appears to have discriminative ability in this population for decreased quality of life and may yield incremental prognostic value beyond global right ventricular ejection fraction assessment, but further study is needed to evaluate methods to limit variability.

Diastolic function and patient-reported quality of life for adolescents and adults with repaired tetralogy of Fallot: a tissue Doppler study.

Left ventricular systolic function is an important indicator of clinical well-being and outcomes for patients with repaired tetralogy of Fallot (TOF). This study tested the hypothesis that left ventricular diastolic function by pulsed-wave tissue Doppler is associated with quality of life in this population. In this study, 38 subjects (age, 31.0 ± 14.1 years) with repaired TOF underwent echocardiogram and completed the Short-Form 36, version 2, a validated quality-of-life assessment, within a median of 0 days (range, 0-90 days). Available cardiovascular magnetic resonance data within 1 year after the echocardiogram were analyzed. The ratio of peak early inflow to peak early annular velocity (E/E') at the lateral mitral annulus correlated inversely with the ability to participate in usual activities without physical limitations (r = -0.37; p = 0.02), whereas the right ventricular diastolic indices were not predictive. The relation of left ventricular diastolic function to quality of life was independent of left ventricular systolic function. This may be related to adverse ventricular-ventricular interactions because lateral mitral E/E' correlated with tricuspid E/E' (r = 0.46; p = 0.008) and the right ventricular myocardial performance index (r = 0.42; p = 0.01). Pulsed-wave tissue Doppler of the mitral annulus is a useful tool in this population and may potentially identify patients in need of intervention before the development of left or right ventricular systolic dysfunction.

Relation of right ventricular dilation, age of repair, and restrictive right ventricular physiology with patient-reported quality of life in adolescents and adults with repaired tetralogy of fallot.

The present study aimed to determine the predictors of patient-reported quality of life and restrictive right ventricular (RV) physiology in adolescents and adults with repaired tetralogy of Fallot. A total of 62 patients (median age 28.5 years, range 14 to 69) undergoing cardiovascular magnetic resonance imaging completed the Short Form 36-item questionnaire, version 2, a validated quality of life assessment. RV inflow curves were generated from the sum of tricuspid inflow and pulmonary insufficiency. The patient-reported quality of life was comparable to population norms. Patients repaired after 1 year of age showed a strong trend toward a greater likelihood of physical component summary age-adjusted z-score ≤-1 (odds ratio 7.50, 95% confidence interval 0.90 to 62.3, p = 0.06). Patients with a RV ejection fraction of <45% reported decreased physical component summary (p = 0.02) and physical functioning (p = 0.02) scores. The RV end-diastolic volume, pulmonary regurgitation, and diastolic indexes did not predict the quality of life. The indexed RV end-diastolic volume was related to diastolic abnormalities, correlating with a greater peak early filling rate (r = 0.71, p <0.0001), ratio of peak early to atrial filling rates (r = 0.45, p = 0.006), and showing a strong trend with the end-diastolic forward flow in the pulmonary trunk (odds ratio 2.67 for moderate dilation and 3.50 for severe dilation, p = 0.06). Patients who underwent repair before 1 year old were more likely to have end-diastolic forward flow (15 of 17 vs 25 of 42, p = 0.03). In conclusion, the RV ejection fraction and age of repair were the best predictors of quality of life in this population, in whom end-diastolic forward flow and associated diastolic parameters appeared to reflect an overdistended ventricle, which might suggest a role for early pulmonary valve replacement.