Caring for the Critically Ill Adult Congenital Heart Disease Patient.

PURPOSE OF REVIEW: This review aims to discuss the unique challenges that adult congenital heart disease (ACHD) patients present in the intensive care unit. RECENT FINDINGS: Recent studies suggest that ACHD patients make up an increasing number of ICU admissions, and that their care greatly improves in centers with specialized ACHD care. Common reasons for admission include arrhythmia, hemorrhage, heart failure, and pulmonary disease. It is critical that the modern intensivist understand not only the congenital anatomy and subsequent repairs an ACHD patient has undergone, but also how that anatomy can predispose the patient to critical illness. Additionally, intensivists should rely on a multidisciplinary team, which includes an ACHD specialist, in the care of these patients.

Trans-apical systemic tricuspid valve-in-ring replacement.

Transcatheter valve replacement offers a safe and effective alternative to traditional surgical techniques in patients with congenital heart disease, especially those at high surgical risk. The most common causes of morbidity and mortality in patients with D-transposition of the great arteries status post Senning or Mustard repair is severe tricuspid valve (TV) regurgitation. Replacement of the systemic TV may be useful in those without severe systemic ventricular dysfunction. We present a case of a patient with D-loop transposition of the great arteries status post Mustard repair and TV ring placement with subsequent severe systemic TV regurgitation, at high surgical risk, who underwent a transcatheter valve replacement via a trans-apical approach using an Edwards Sapien XT valve.

Impact of Right-Sided-Catheter-Based Valve Implantation on Decision-Making in Congenital Heart Disease.

There is a growing appreciation for the adverse long-term impact of right-sided valvular dysfunction in patients with congenital heart disease. Although right-sided valvular stenosis and/or regurgitation is often better tolerated than left-sided valvular dysfunction in the short and intermediate term, the long-term consequences are numerous and include, but are not limited to, arrhythmias, heart failure, and multi-organ dysfunction. Surgical right-sided valve interventions have been performed for many decades, but the comorbidities associated with multiple surgeries are a concern. Transcatheter right-sided valve replacement is safe and effective and is being performed at an increasing number of centers around the world. It offers an alternative to traditional surgical techniques and may potentially alter the decision making process whereby valvular replacement is performed prior to the development of long-term sequelae of right-sided valvular dysfunction.

Transcatheter management of obstructed baffle repairs of partial anomalous pulmonary veins: a case series.

Background: Partial anomalous pulmonary venous return (PAPVR) can be surgically corrected using a pericardial baffle. This baffle can become obstructed post-surgery, leading to pulmonary hypertension and right heart dysfunction if not detected and corrected. Case summary: We describe three patients with occluded PAPVR baffles who underwent drug-coated balloon angioplasty and stenting of the obstructed baffle. In each case, baffle obstruction was detected post-operatively on surveillance cross-sectional imaging, and an invasively measured pulmonary capillary wedge-to-left atrium gradient was noted to be elevated. Post-intervention, each patient had an improvement in baffle flow by angiography as well as lung perfusion as assessed by nuclear medicine scintigraphy. Discussion: Given the subtle symptomatology of obstructed PAPVR pericardial baffle repairs, surveillance imaging is necessary to detect occluded baffles and intervene before downstream right heart disease and pulmonary hypertension develops. Given the high rates of re-stenosis in pulmonary vein stenting, pre-treatment of occluded PAPVR baffles with drug-coated balloons may help reduce re-intervention rates.

Anomalous Aortic Origin of a Coronary Artery.

BACKGROUND: Although anomalous aortic origin of a coronary artery (AAOCA) is associated with risk of sudden cardiac arrest (SCA), there is a spectrum of disease, with the appropriate management for many remaining unclear. Increasing data warrant review for an updated perspective on management. METHODS: A panel of congenital cardiac surgeons, cardiologists, and imaging practitioners reviewed the current literature related to AAOCA and its management. Survey of relevant publications from 2010 to the present in PubMed was performed. RESULTS: The prevalence of AAOCA is 0.4% to 0.8%. Anomalous left coronary artery is 3 to 8 times less common than anomalous right coronary, but carries a much higher risk of SCA. Nevertheless, anomalous right coronary is not completely benign; 10% demonstrate ischemia, and it remains an important cause of SCA. Decision-making regarding which patients should be recommended for surgical intervention includes determining anatomic features associated with ischemia, evidence of ischemia on provocative testing, and concerning cardiovascular symptoms. Ischemia testing continues to prove challenging with low sensitivity and specificity, but the utility of new modalities is an active area of research. Surgical interventions focus on creating an unobstructed path for blood flow and choosing the appropriate surgical technique given the anatomy to accomplish this. Nontrivial morbidity has been reported with surgery, including new-onset ischemia. CONCLUSIONS: A proportion of patients with AAOCA demonstrate features and ischemia that warrant surgical intervention. Continued work remains to improve the ability to detect inducible ischemia, to risk stratify these patients, and to provide guidance in terms of which patients warrant surgical intervention.

Lifetime Management of Adolescents and Young Adults with Congenital Aortic Valve Disease.

In this invited expert review, we focus on evolving lifetime management strategies for adolescents and young adults with congenital aortic valve disease, acknowledging that these patients often require multiple interventions during their lifetime. Our goal is to preserve the native aortic valve when feasible. Leveraging advanced multimodality imaging, a detailed assessment of the aortic valve and root complex can be obtained, and a surgically approach tailored to an individual patient's anatomy and pathology can be used. In turn, aortic valve repair and reconstruction can be offered to a greater number of patients, either as a definitive strategy or as a component of a staged strategy to delay the need for aortic valve replacement until later in life when more options are available.

Transcatheter Aortic Valve Replacement in Congenital Heart Disease.

Transcatheter aortic valve replacement is not widely used in patients with congenital heart disease. We describe our single-center experience of transcatheter aortic valve replacement in congenital heart disease, demonstrating short-term feasibility and safety, role in lifetime management of congenital aortic valve disease, and use as a bridge to recovery, future surgery, or transplantation.

Efficacy of Drug-Coated Balloon Angioplasty in Pulmonary Vein Stenosis or Total Occlusion.

BACKGROUND: Current therapies for pulmonary vein stenosis (PVS) or pulmonary vein total occlusion (PVTO) involving angioplasty and stenting are hindered by high rates of restenosis. OBJECTIVES: This study compares a novel approach of drug-coated balloon (DCB) angioplasty and stenting with the current standard of care in PVS or PVTO due to pulmonary vein isolation (PVI). METHODS: A retrospective single-center study analyzed patients with PVS or PVTO due to PVI who underwent either angioplasty and stenting (NoDCB group; December 2012-December 2016) or DCB angioplasty and stenting (DCB group; January 2018-January 2021). Multivariable Andersen-Gill regression analysis assessed the risk of restenosis and target lesion revascularization (TLR). RESULTS: The NoDCB group comprised 58 patients and 89 veins, with a longer median follow-up of 35 months, whereas the DCB group included 26 patients and 33 veins, with a median follow-up of 11 months. The DCB group exhibited more PVTO (NoDCB: 12.3%; DCB: 42.4%; P = 0.0001), with a smaller reference vessel size (NoDCB: 10.2 mm; DCB: 8.4 mm; P = 0.0004). Follow-up computed tomography was performed in 82% of NoDCB and 85% of DCB, revealing lower unadjusted rates of restenosis (NoDCB: 26%; DCB: 14.3%) and TLR (NoDCB: 34.2%; DCB: 10.7%) in the DCB group. DCB use was associated with a significantly lower risk of restenosis and TLR (HR: 0.003: CI: 0.00009-0.118; P = 0.002). CONCLUSIONS: The novel approach of DCB angioplasty followed by stenting is effective and safe and significantly reduces the risk of restenosis and reintervention compared with the standard of care in PVS or PVTO due to PVI.

Intravascular Lithotripsy for Severe RVOT Calcification to Optimize Transcatheter Pulmonary Valve Replacement.

The presence of severe right ventricular outflow tract calcification may preclude safe and effective transcatheter pulmonary valve replacement in patients with pulmonary allograft stenosis owing to the risk of conduit tear and suboptimal annular expansion. Debulking calcium using intravascular lithotripsy within the right ventricular outflow tract may mitigate this risk and improve valve hemodynamics. (Level of Difficulty: Advanced.).

Concomitant Mitral Valve Re-Repair for Mixed Congenital and Degenerative Mitral Disease, Tricuspid Repair, and Cox-Maze Procedure in a College Athlete.

A significant proportion of patients undergoing repair of congenital mitral valve disease will require a subsequent reoperation. During somatic growth, mitral valve repair is preferable to replacement as it allows for annular growth, preservation of ventricular function, and avoidance of lifelong anticoagulation. Techniques to facilitate successful re-repairs for congenital and non-rheumatic mixed degenerative mitral valve disease are not well-described in the literature. Description of the encountered pathology and surgical maneuvers utilized in this case provides real-world tools to help surgeons deal with limited orifice availability, fibrosis, and multilevel lesions. We describe a mitral valve re-repair in a young athlete for a rare cleft posterior mitral leaflet, with a simultaneous tricuspid valve repair and Cox-Maze procedure. We focus on technical pearls that address specific anatomic challenges within our surgical approach.

Multifocal disease progression and subsequent intervention in patients with actin alpha-2 variants: A single-center experience.

OBJECTIVES: To describe patient characteristics and indications for surgical intervention, reoperation, and outcomes in patients with actin alpha-2 (ACTA2) variants. METHODS: A single-center retrospective cohort study with prospective follow-up was performed for 38 patients with an ACTA2 variant. RESULTS: From 1999 to 2020, 26 (70%) patients underwent surgery; 11 remain under surveillance (mean follow-up, 7.5 ± 5 years). Median age at index operation was 42 (range, 10-69) years, with 4 pediatric cases. Thoracic aortic aneurysm was present in 19 (73%) patients (mean adult max diameter, 5.2 ± 0.8 cm; pediatric z score, 10.7 ± 5.4). Aortic dissection was present in 13 (50%) patients, with 4 (15%) having type A dissection. Operations included replacement of the aortic root in 16 (17%), ascending aorta in 20 (77%), and aortic arch in 14 (54%) patients. Four (15%) patients had coronary artery disease, and 2 (7.7%) underwent concomitant coronary artery bypass grafting. There was no operative mortality, stroke, reoperation for bleeding, or dialysis-dependent renal failure; One (3.8%) patient developed acute on chronic kidney injury. Three patients (12%) required prolonged ventilation. Eleven (42%) patients underwent 26 reoperations, median time 45 (range, 4-147) months, including 5 open thoracoabdominal aneurysm repairs. CONCLUSIONS: Patients with ACTA2 variants frequently develop aortic aneurysm and are at risk of aortic dissection and coronary artery disease. However, age at diagnosis and symptoms at presentation are highly variable. Multiple operations are often required for disease management, particularly after dissection. Close monitoring and timely intervention are important in mitigating disease progression and improving outcomes.

The Added Value of Preprocedural Cardiac Computed Tomography in Planning Left Atrial Appendage Closure With the Watchman FLX Device.

•Cardiac computed tomography (CCT) can be used as a useful complementary tool in preprocedural planning of left atrial appendage closure.•CCT planning includes assessing exclusion criteria, device sizing, sheath selection, anticipating challenging anatomies, and transseptal puncture planning.•Procedural 3D intracardiac echocardiography (3D-ICE) is increasingly being used instead of transesophageal echocardiography. In this setting, preprocedural CCT is useful to compensate for 3D ICE's reduced resolution.

Characteristics and immediate outcomes of patients who underwent percutaneous balloon mitral valvuloplasty at the Jakaya Kikwete Cardiac Institute, Tanzania.

BACKGROUND: For rheumatic mitral stenosis (MS), a multidisciplinary evaluation is mandatory to determine the optimal treatment: medical, percutaneous balloon mitral valvuloplasty (PBMV) or valve surgery. Clinical and imaging evaluations are essential for procedural risk assessment and outcomes. PBMV interventions are increasingly available in Africa and are feasible options for selected candidates. Enhancing PBMV training/skills transfer across most of African countries is possible. OBJECTIVES: The aim of this study was to provide insight into the clinical practice of patients with rheumatic MS evaluated for PBMV in a Tanzanian teaching hospital and to define the role of imaging, and evaluate the heart team and training/skills transfer in PBMV interventions. METHODS: From August 2019 to May 2022, 290 patients with rheumatic MS were recruited consecutively in the Tanzania Mitral Stenosis study. In total, 43 (14.8%) patients were initially evaluated for eligibility for PBMV by a heart team. We carried out the clinical assessment, laboratory investigations, transthoracic/oesophageal echocardiography (TTE/TEE) and electrocardiography. RESULTS: The median age was 31 years (range 11-68), and two-thirds of the patients were female (four diagnosed during pregnancy). Two patients had symptomatic MS at six and eight years. Nine patients had atrial fibrillation with left atrial thrombus in three, and two were detected by TEE. Nine patients in normal sinus rhythm had spontaneous echo contrast. The mean Wilkins score was 8.6 (range 8-12). With re-evaluation by the local and visiting team, 17 patients were found to have unfavourable characteristics: Bi-commissural calcification (four), ≥ grade 2/4 mitral regurgitation (six), high scores and left atrial thrombus (three), left atrial thrombus (two), and severe pulmonary hypertension (two). Three patients died before the planned PBMV. Eleven patients were on a waiting list. We performed PBMV in 12 patients, with success in 10 of these, and good short-term outcomes [mean pre-PBMV (16.03 ± 5.52 mmHg) and post-PBMV gradients (3.08 ± 0.44 mmHg, p < 0.001)]. There were no complications. CONCLUSIONS: PBMV had good outcomes for selected candidates. TEE is mandatory in pre-PBMV screening and for procedural guidance. In our cohort, patients with Wilkins score of up to 11 underwent successful PBMV. We encourage PBMV skills expansion in low- and middle-income countries, concentrating on expertise centres.

Transcatheter Valve Implantation in Reversed Potts Shunt in Pulmonary Arterial Hypertension: Keeping the Shunt Reversed.

The reversed Potts shunt is designed to offload the right ventricle in severe pulmonary arterial hypertension. We present a case of bidirectional flow across a reversed Potts shunt leading to pulmonary edema, with clinical improvement after implantation of a transcatheter valve in the shunt to maintain unidirectional flow. (Level of Difficulty: Advanced.).

Multidisciplinary Approach to Isolated Pulmonary Vein Compression by an Enlarging Vertebral Osteophyte.

Compression of mediastinal structures by vertebral osteophytes is rare. We report a case of pulmonary vein compression by a vertebral osteophyte that failed stenting. A minimally invasive approach to osteophyte removal with subsequent re-expansion angioplasty yielded an optimal outcome, negating the need for cardiopulmonary bypass, stent removal, and pulmonary venoplasty. (Level of Difficulty: Intermediate.).

Patient-specific fluid-structure simulations of anomalous aortic origin of right coronary arteries.

Objectives: Anomalous aortic origin of the right coronary artery (AAORCA) may cause ischemia and sudden death. However, the specific anatomic indications for surgery are unclear, so dobutamine-stress instantaneous wave-free ratio (iFR) is increasingly used. Meanwhile, advances in fluid-structure interaction (FSI) modeling can simulate the pulsatile hemodynamics and tissue deformation. We sought to evaluate the feasibility of simulating the resting and dobutamine-stress iFR in AAORCA using patient-specific FSI models and to visualize the mechanism of ischemia within the intramural geometry and associated lumen narrowing. Methods: We developed 6 patient-specific FSI models of AAORCA using SimVascular software. Three-dimensional geometries were segmented from coronary computed tomography angiography. Vascular outlets were coupled to lumped-parameter networks that included dynamic compression of the coronary microvasculature and were tuned to each patient's vitals and cardiac output. Results: All cases were interarterial, and 5 of 6 had an intramural course. Measured iFRs ranged from 0.95 to 0.98 at rest and 0.80 to 0.95 under dobutamine stress. After we tuned the distal coronary resistances to achieve a stress flow rate triple that at rest, the simulations adequately matched the measured iFRs (r = 0.85, root-mean-square error = 0.04). The intramural lumen remained narrowed with simulated stress and resulted in lower iFRs without needing external compression from the pulmonary root. Conclusions: Patient-specific FSI modeling of AAORCA is a promising, noninvasive method to assess the iFR reduction caused by intramural geometries and inform surgical intervention. However, the models' sensitivity to distal coronary resistance suggests that quantitative stress-perfusion imaging may augment virtual and invasive iFR studies.

Coronary artery disease in adults with anomalous aortic origin of a coronary artery.

Objectives: This study sought to characterize coronary artery disease (CAD) among adults diagnosed with an anomalous aortic origin of a coronary artery (AAOCA). We hypothesized that coronaries with anomalous origins have more severe CAD stenosis than coronaries with normal origins. Methods: This single-center study of 763 adults with AAOCA consisted of 620 patients from our cardiac catheterization database (1958-2009) and 273 patients from electronic medical records query (2010-2021). Within left main, anterior descending, circumflex, and right coronary arteries, the CAD stenosis severity, assessed by invasive or computer tomography angiography, was modeled with coronary-level variables (presence of an anomalous origin) and patient-level variables (age, sex, comorbidities, and which of the four coronaries was anomalous). Results: Of the 763 patients, 472 (60%) had obstructive CAD, of whom, 142/472 (30%) had obstructive CAD only in the anomalous coronary. Multivariable modeling showed similar CAD stenosis severity between coronaries with anomalous versus normal origins (P = .8). Compared with AAOCA of other coronaries, the anomalous circumflex was diagnosed at older ages (59.7 ± 11.1 vs 54.3 ± 15.8 years, P < .0001) and was associated with increased stenosis in all coronaries (odds ratio, 2.7; 95% confidence interval, 2.2-3.4, P < .0001). Conclusions: Among adults diagnosed with AAOCA, the anomalous origin did not appear to increase the severity of CAD within the anomalous coronary. In contrast to the circumflex, AAOCA of the other vessels may contribute a greater ischemic burden when they present symptomatically at younger ages with less CAD. Future research should investigate the interaction between AAOCA, CAD, and ischemic risk to guide interventions.

Interaction Between Race and Income on Cardiac Outcomes After Percutaneous Coronary Intervention.

Background Compared with White Americans, Black Americans have a greater prevalence of cardiac events following percutaneous coronary intervention. We evaluated the association between race and neighborhood income on post-percutaneous coronary intervention cardiac events and assessed whether income modifies the effect of race on this relationship. Methods and Results Consecutive patients (n=23 822) treated with percutaneous coronary intervention from January 1, 2000, to December 31, 2016, were included. All-cause mortality and major adverse cardiac event were assessed at 3 years. Extended 10-year follow-up was performed for those residing locally (n=1285). Neighborhood income was derived using median adjusted annual gross household income reported within the patient's zip code. We compared differences in treatment and outcomes, adjusting for race, income, and their interaction. In total, 3173 (13.3%) patients self-identified as Black Americans, and 20 649 (86.7%) self-identified as White Americans. Black Americans had a worse baseline cardiac risk profile and lower neighborhood income compared with White Americans. Although risk profile improved with increasing income in White Americans, no difference was observed across incomes among Black Americans. Despite similar long-term outpatient cardiology follow-up and medication prescription, risk profiles among Black Americans remained worse. At 3 years, unadjusted all-cause mortality (18.0% versus 15.2%; P<0.001) and major adverse cardiac event (37.3% versus 34.6%; P<0.001) were greater among Black Americans and with lower income (both P<0.001); race, income, and their interaction were not significant predictors in multivariable models. At 10-year follow-up, increasing income was associated with improved outcomes only in White Americans but not Black Americans. In multivariable models for major adverse cardiac event, income (hazard ratio [HR], 0.97 [95% CI, 0.96-0.98]; P=0.005), Black race (HR, 1.77 [95% CI, 1.58-1.96]; P=0.006), and their interaction (HR, 0.98 [95% CI, 0.97-0.99]; P=0.003) were significant predictors. Similar findings were observed for cardiac death. Conclusions Early 3-year post-percutaneous coronary intervention outcomes were driven by worse risk factor profiles in both Black Americans and those with lower neighborhood income. However, late 10-year outcomes showed an independent effect of race and income, with improving outcomes with greater income limited to White Americans. These findings illustrate the importance of developing novel care strategies that address both risk factor modification and social determinants of health to mitigate disparities in cardiac outcomes.

Adiponectin mediates cardioprotection in oxidative stress-induced cardiac myocyte remodeling.

Reactive oxygen species (ROS) induce matrix metalloproteinase (MMP) activity that mediates hypertrophy and cardiac remodeling. Adiponectin (APN), an adipokine, modulates cardiac hypertrophy, but it is unknown if APN inhibits ROS-induced cardiomyocyte remodeling. We tested the hypothesis that APN ameliorates ROS-induced cardiomyocyte remodeling and investigated the mechanisms involved. Cultured adult rat ventricular myocytes (ARVM) were pretreated with recombinant APN (30 µg/ml, 18 h) followed by exposure to physiologic concentrations of H(2)O(2) (1-200 µM). ARVM hypertrophy was measured by [(3)H]leucine incorporation and atrial natriuretic factor (ANF) and brain natriuretic peptide (BNP) gene expression by RT-PCR. MMP activity was assessed by in-gel zymography. ROS was induced with angiotensin (ANG)-II (3.2 mg·kg(-1)·day(-1) for 14 days) in wild-type (WT) and APN-deficient (APN-KO) mice. Myocardial MMPs, tissue inhibitors of MMPs (TIMPs), p-AMPK, and p-ERK protein expression were determined. APN significantly decreased H(2)O(2)-induced cardiomyocyte hypertrophy by decreasing total protein, protein synthesis, ANF, and BNP expression. H(2)O(2)-induced MMP-9 and MMP-2 activities were also significantly diminished by APN. APN significantly increased p-AMPK in both nonstimulated and H(2)O(2)-treated ARVM. H(2)O(2)-induced p-ERK activity and NF-κB activity were both abrogated by APN pretreatment. ANG II significantly decreased myocardial p-AMPK and increased p-ERK expression in vivo in APN-KO vs. WT mice. ANG II infusion enhanced cardiac fibrosis and MMP-2-to-TIMP-2 and MMP-9-to-TIMP-1 ratios in APN-KO vs. WT mice. Thus APN inhibits ROS-induced cardiomyocyte remodeling by activating AMPK and inhibiting ERK signaling and NF-κB activity. Its effects on ROS and ultimately on MMP expression define the protective role of APN against ROS-induced cardiac remodeling.