Biventricular longitudinal strain analysis using CMR feature-tracking: prognostic value in Eisenmenger syndrome.

BACKGROUND: Evidence to support risk stratification in Eisenmenger syndrome (ES) is still very limited. We hypothesized that biventricular longitudinal strain analysis could have potential prognostic value in ES. METHODS: We prospectively enrolled fifty-seven consecutive ES patients with post-tricuspid shunt who underwent both cardiovascular magnetic resonance (CMR) and right heart catheterization between June 2013 and March 2022. Biventricular longitudinal strains were evaluated by CMR feature-tracking analysis. The composite endpoint included all-cause mortality and re-admission for heart failure or hemoptysis. Cox regression analysis, Kaplan-Meier curves, and C-index were employed to assess the relationship between biventricular longitudinal strain and prognosis. RESULTS: During a median follow-up of 33 months (interquartile range: 12-50), 20 (35.1%) patients reached the composite endpoint. Patients with composite endpoints had significantly lower absolute values of left ventricular global longitudinal strain (LV GLS) and right ventricular free wall longitudinal strain (RV FWLS) than patients without composite endpoints (p < 0.05). Multivariate Cox regression analysis demonstrated that LV GLS and RV FWLS were independent predictors for composite endpoints (hazard ratio [HR]: 1.37, 95% confidence interval [CI]: 1.08-1.75, p = 0.010 and HR: 1.19, 95% CI: 1.01-1.41, p = 0.042). Kaplan-Meier analysis indicated that patients with both lower absolute values of LV GLS and RV FWLS were more likely to be at an even higher risk of composite endpoints (p < 0.001). Furthermore, the combined addition of LV GLS and RV FWLS provided incremental value for the prognostic model including clinical parameters and biventricular ejection fraction (C-index increased from 0.75 to 0.86, p = 0.004). CONCLUSION: Impaired biventricular longitudinal strains improved prognostic prediction of ES patients with post-tricuspid shunt.

Atrial Septal Defect (ASD) Repair Unveiling an Unusual Conduction Conundrum: A Wenckebach Case Report.

Atrial septal defects are a common congenital malformation that can lead to an elevated risk for stroke due to the bypass of the lungs by deep vein thrombosis, as well as the expected repercussions of pulmonary hypertension if left untreated. Surgical intervention is definitive; however, recent advancements in treatment options, such as percutaneous intervention, represent a safer and equally effective way to treat this congenital complication. While safer, percutaneous interventions can also lead to adverse events that may force patients to present to the emergency department. Here, we present a unique case of a patient with congenital atrial septal defect status post-percutaneous intervention who developed a new-onset second-degree AV block, Mobitz type 1 Wenckebach rhythm.

Successful Cardiac Surgical Management in a Trisomy 21 Child After Long-Term Hospitalization Associated With Bronchopneumonia and Hepatitis C Virus Seropositivity: A Case Report.

A 31-month-old girl with trisomy 21 (Down syndrome) was seen in the emergency department of pediatrics because of oxygen desaturation associated with features of lower respiratory tract infections. She was born at full term and diagnosed with congenital heart disease (CHD) having ventricular septal defect (VSD), and patent ductus arteriosus (PDA); consequently, she underwent corrective surgery after adequate optimization of treatment. Incidentally, she was detected to have the presence of anti-hepatitis C virus (HCV) antibodies. In this case report, we mainly focus on the multi-modal approach to medical and surgical management.

How severity and classification of pulmonary hypertension affect pregnancy outcomes: a systematic review and timeline.

Women with pulmonary hypertension (PH) have increased mortality during pregnancy and the peripartum period. An increasing number of publications suggest improvements in maternal outcomes, so we conducted a systematic review focusing on disease severity and maternal survival. After screening 9097 potential studies from 1967 to 2021, we identified 66 relevant publications. Outcomes improved continuously over time and mortality fell from 11.6% in studies published before 2015 to 8.2% in studies published after 2015. Mortality was lower in patients with mild disease (0.8%) than in those with Eisenmenger syndrome (26.2%) or idiopathic pulmonary arterial hypertension (7.4-24.0%). One major drawback of the published studies is that they define severity using echocardiographic-estimated pulmonary artery pressures, without considering more contemporary parameters. This systematic review provides new insights for preconception counseling on pregnancy risks related to PH and suggests that PH classification and severity should be carefully considered in determining an individual's pregnancy-associated risk.

Complex Cardiovascular Morbidities in Prader-Willi Syndrome: A Multidisciplinary Approach.

This case emphasizes the complexity of Prader-Willi syndrome (PWS), the need for a collaborative approach from specialists, and a closer look at the various cardiovascular complexities associated with this syndrome. While current treatments focus on managing symptoms, ongoing genetic research offers hope for more favorable outcomes. Further studies are crucial to gauge the effectiveness of these treatments for PWS patients. We detail a patient with a complex medical history of PWS, further complicated by congenital heart disease with Eisenmenger's syndrome, diabetes mellitus, pulmonary hypertension, venous insufficiency, hypothyroidism, and hyperlipidemia. Reported in this study is a compilation of clinical data as well as suggestions from several medical specialists in applying a multifaceted approach to treatment, significantly emphasizing the need for interdisciplinary care and management of patients experiencing a combination of various medical issues with an emphasis on cardiovascular complications.

Medical Therapy and Monitoring in Adult Congenital Heart Disease Heart Failure.

Heart failure (HF) in adult congenital heart disease (ACHD) is an increasingly common problem facing ACHD and advanced heart disease and transplant providers. Patients are highly nuanced, and therapies are poorly studied. Standard HF medications are often used in patients who are not targets of large clinical trials. HF management in this data-free zone requires focused, comprehensive team-based care and close follow-up and communication with patients.

Cyanotic Nephropathy in an Adult Patient with Eisenmenger Syndrome: A Case Report and Literature Review.

INTRODUCTION: Cyanotic nephropathy, a rare disease characterized by proteinuria, decreased estimated glomerular filtration rate, thrombocytopenia, polycythemia, and hyperuricemia, may occasionally be secondary to cyanotic congenital heart disease (CHD). There are currently no detailed diagnostic criteria or treatments for cyanotic nephropathy, owing to its extremely low incidence. Eisenmenger syndrome (ES) was initially defined by Paul Wood in pathophysiologic terms as "pulmonary hypertension (PH) at the systemic level, caused by a high pulmonary vascular resistance, with a reversed or bidirectional shunt at the aorto-pulmonary, ventricular, or atrial level." It typically develops in the presence of large, unrepaired atrial or ventricular septal defects, arterial shunts, or complex forms of CHD and is the most severe hemodynamic phenotype of pulmonary arterial hypertension associated with CHD. This study aimed to outline the case of an ES patient who developed cyanotic nephropathy and successfully achieved clinical remission through primary disease treatment and symptomatic management. Overall, this case expands our understanding of cyanotic nephropathy and lays a theoretical reference for the treatment of ES. CASE PRESENTATION: A 33-year-old Chinese female attended the outpatient department with abnormal urine test results over the past two and a half years. Following a comprehensive medical history collection, she underwent the necessary tests. Cardiac color ultrasound displayed a significant widening of the pulmonary artery and PH (severe), as well as mild tricuspid regurgitation and patent ductus arteriosus. The results of the kidney biopsy, combined with clinical findings, suggested a high risk of polycythemia-related kidney disease. She was eventually diagnosed with cyanotic nephropathy and ES. Her symptoms were relieved following symptomatic treatment, such as the administration of ambrisentan, febuxostat, and home oxygen therapy. Her follow-up visit at 6 months demonstrated improvements in hyperuricemia and a significant increase in physical strength. CONCLUSION: Cyanotic nephropathy is a rare condition in adults. Kidney biopsy remains the gold standard of diagnosis for various nephropathies. Active treatment of CHD and alleviating hypoxia may be pivotal for the treatment of cyanotic nephropathy.

Successful intraoperative management of laparoscopic hysterectomy in a patient with Eisenmenger syndrome: a case report.

BACKGROUND: Patients with Eisenmenger syndrome (ES) requiring noncardiac surgery are at a significantly high risk of perioperative morbidity and mortality. However, perioperative management of patients with ES requiring laparoscopic surgery remains unclear. CASE PRESENTATION: We describe the case of a patient with ES who underwent laparoscopic hysterectomy under general anesthesia with a peripheral nerve block. The objectives of the perioperative management included the following: (1) maintaining systemic vascular resistance and cardiac output through euvolemia, facilitated by the infusion of noradrenaline, and (2) preventing a reduction in oxygen-carrying capacity and factors that elevate pulmonary vascular resistance, such as pain, hypoxia, and decreased body temperature. Although laparoscopic procedures involved an increased risk in patients with ES, they are less invasive than open surgeries. CONCLUSION: This report describes the successful anesthetic management of a patient with ES, ensuring a balance between systemic and pulmonary vascular resistance.

The Anesthetic Complexity of Eisenmenger Syndrome: A Clinical Case.

Eisenmenger syndrome (ES) is a complex, multisystemic, and rare clinical entity, given that currently, most congenital heart diseases can be corrected in childhood. The high anesthetic risk in these patients poses a challenge for anesthesiology. There are few cases described in the literature of anesthetic approaches using ketamine and dexmedetomidine in ES cases, particularly under Monitored Anesthesia Care (MAC). We describe the clinical case of a 40-year-old patient with trisomy 21, intellectual disability, and ES secondary to a single atrioventricular (AV) valve, scheduled for cranial magnetic resonance imaging (MRI) under sedation due to a suspected space-occupying lesion. Sedation was performed under MAC with dexmedetomidine and ketamine. The procedure proceeded without complications. The anesthetic approach in ES patients, given the clinical complexity, requires planning by a multidisciplinary team and should be tailored to the procedure and its duration.

Real-world diagnostic landscape and incidence of pulmonary hypertension in adult congenital heart disease patients using administrative claims data in Japan.

BACKGROUND: Although pulmonary hypertension (PH) and Eisenmenger's syndrome (ES) are common complications in adult congenital heart disease (ACHD), the frequency of diagnostic tests and the incidence of PH/ES in patients with ACHD in Japanese clinical practice are unclear. Therefore, we sought to clarify the frequency of diagnostic tests and incidence of PH/ES in patients with ACHD using the Medical Data Vision (MDV) database, the largest anonymized database of diagnosis procedure combination hospitals in Japan. METHODS: We conducted a retrospective cohort study using the MDV database (April 2008 to December 2021) of patients with ACHD (International Classificaiton of Diseases, 10th revision codes: Q203-204, Q210-213, Q250) aged ≥15 years. The frequency of laboratory/clinical tests and the incidence of PH/ES were calculated. Subgroup analyses were performed for the periods 2008-2015 and 2016-2021. RESULTS: Overall, 28219 ACHD patients were extracted from the MDV database (females 56.3%, males 43.7%; mean ± standard deviation age 44.7 ± 23.5 years). The mean ± standard deviation follow-up period was 2.5 ± 2.7 years. The frequencies of electrocardiography, ultrasonography, brain natriuretic peptide (BNP), N-terminal pro-BNP (NT-proBNP), right heart catheterization, and pulmonary function tests (DLCO) were 2149.8, 1054, 1233, 340, 40.0, and 6.0 per 1000 person-years, respectively. The incidence rate of PH/ES was 32.8 per 1000 person-years. The incidence rate of PH/ES increased from 24.6 to 46.7 per 1000 person-years from 2008-2015 to 2016-2021. CONCLUSION: We have clarified the frequency of diagnostic tests related to PH/ES and the incidence of PH/ES in patients with ACHD in clinical practice in Japan, including non-specialist institutions for PH.

Remimazolam in perioperative management of Eisenmenger syndrome: a case report.

BACKGROUND: Eisenmenger syndrome (ES) is characterized by severe and irreversible pulmonary hypertension stemming from an uncorrected intracardiac shunt of significant size. The imbalance between systemic and pulmonary artery pressures predisposes patients with ES to the risk of cardiac arrest. Remimazolam has caused less circulatory depression, which may be advantageous for ES. However, no studies reported the use of remimazolam in perioperative ES management. CASE PRESENTATION: A 45-year-old female patient with ES derived from a ventricular septal defect was scheduled to undergo bilateral adnexectomy for an ovarian tumor. Her oxygen saturation was 80% with 3 L/min oxygen at rest, and her pulmonary and systemic flow ratio was 0.53. She underwent general anesthesia with remimazolam, and intraoperative hemodynamics was stable without hypotension or reduced oxygen saturation. CONCLUSIONS: Our successful management of ovarian tumor surgery in a patient with ES using remimazolam reveals its potential effectiveness in perioperative care.

Management of Unrepaired Tetralogy of Fallot in an 86-Year-Old Patient Undergoing Transcatheter Aortic Valve Replacement.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect, typically requiring early treatment in infancy. Untreated TOF is associated with poor survival, with most uncorrected patients not surviving beyond the third decade. Here, we present a unique case of an 86-year-old female with uncorrected TOF who underwent a transcatheter aortic valve replacement (TAVR) procedure due to severe aortic stenosis (AS). The patient's TOF was identified during infancy, and she was categorized as an acyanotic "pink baby." Notably, the first palliative surgery for TOF was performed in 1944, when the patient was seven years old. The patient never underwent corrective surgery for TOF and continued to lead a symptom-free life until she developed severe AS later in life. The TAVR procedure significantly improved her symptoms, illustrating the importance of considering alternative etiologies for symptoms in elderly patients with uncorrected TOF and AS. In addition, we underscore the anesthetic management during TAVR, specifically highlighting the challenges addressed, such as the utilization of general anesthesia with transesophageal echocardiography (TEE) and the placement of a pulmonary artery (PA) catheter.

Application of a modified clinical classification for pulmonary arterial hypertension associated with congenital heart disease in children: emphasis on atrial septal defects and transposition of the great arteries. An analysis from the TOPP registry.

Aims: A proportion of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) do not fit in the current classification. We aimed to analyse the applicability of an adapted clinical classification of PAH-CHD to pediatric patients using the TOPP-1 registry (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) and focus on atrial septal defects (ASD) and transposition of the great arteries (TGA). Methods and results: Hemodynamic and clinical data of all patients with PAH-CHD in the TOPP cohort were reviewed. Patients were classified according to predefined ABCDE categories (A: Eisenmenger syndrome, B: left-to-right shunt, C: coincidental defects, including all ASDs, D: corrected CHD, E: TGA), or as complex CHD (group 5), by 2 independent investigators. In case of disagreement, a third reviewer could either settle a final decision, or the patient was deemed not classifiable. Survival curves were calculated for each group and compared to idiopathic PAH patients of the registry. A total of 223 out of 531 patients in the registry had PAH-CHD, and 193 were categorized to the following groups: A 39(20%), B 27(14%), C 62(32%) including 43 ASDs, D 58(30%), E 7(4%), whereas 6 patients were categorized as group 5, and 10 patients were unable to be classified. No survival difference could be demonstrated between the groups. Conclusions: This modified classification seems to be more applicable to pediatric PAH-CHD patients than the previous classification, but some patients with PAH-CHD who never had a shunt remain unclassifiable. The role of ASD in pediatric PH should be reconsidered.

The Unrepaired Tetralogy of Fallot: A Tale of Delayed Presentation and Limited Access to Care.

Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease characterized by four distinct anatomical features. While surgical repair has significantly improved long-term outcomes, some individuals, particularly those from low socioeconomic backgrounds who lack access to medical care, may suffer from complications such as pulmonary hypertension (pHTN) and heart failure. We present a case report of a young female with unrepaired TOF who presented with acute-on-chronic hypoxic respiratory failure and heart failure, highlighting the complex nature and challenges associated with this condition.

Prognosis factors in heart-lung transplantation: 30 years experience in a reference center.

INTRODUCTION: Heart-lung transplantation has shown a progressive decrease in the number of procedures. There is a lack of information about this field in Spain. The main goal of this study is to analyze the experience of a national reference hospital. METHODS: We performed a retrospective study of a historical cohort of heart-lung transplanted patients in a single center, during a 30 years period (from 1990 to 2021). The associations between variables were evaluated using the χ2 test or Fisher's exact test. Survival was analyzed using the Kaplan-Meier method. Differences were evaluated using the log-rank test and multivariate analysis with the Cox method. RESULTS: A decrease in the number of procedures performed in the last decade was observed [2000-2009: 19 procedures (44.2%); 2010-2021: 15 procedures (34.8%)]. Early postoperative mortality was 23.3%, falling to 13.3% from 2010. In-hospital mortality was 41%, falling to 33% from 2010. Main factors related to higher mortality: previous thoracic surgery, corticosteroid therapy, extracorporeal circulation (ECLS) greater than 200 min, ischemia time greater than 300 min, and tracheal dehiscence (p < 0.005). Overall survival at one, five, and ten years was 58%, 44.7%, and 36.1%, respectively. Factors associated with lower survival rates: previous thoracic surgery, male donor, extracorporeal circulation greater than 200 min, ischemia time greater than 300 min, tracheal dehiscence and weight difference (p < 0.005). CONCLUSIONS: There has been a progressive decrease in the number of heart-lung transplantations, being more evident in the last decade, but showing an improvement in both mortality and survival.

One-minute sit-to-stand test in patients with pulmonary arterial hypertension associated with congenital heart disease: A single-center prospective study.

BACKGROUND: Patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) have reduced exercise capacity. Recently, the 1-minute sit-to-stand test (1MSTST), which measures the number of repetitions from sitting to standing position in 1 minute, has been proposed as an alternative test to the 6-minute walking test (6MWT). The aim of our study was to assess the safety and results of the 1MSTST in comparison to the 6MWT in patients with PAH-CHD. METHODS: Consecutive adult patients with PAH-CHD underwent the 6MWT and the 1MSTST on the same day. The 6-minute walking distance in meters and the number of repetitions on the 1MSTST were measured. Heart rate, peripheral oxygen saturations, Borg dyspnea score, and lower limb fatigue were recorded before and immediately after testing. Correlations between both tests and clinical, laboratory, and imaging parameters were statistically analyzed. RESULTS: The study included 40 patients (50% female, mean age 43 ± 15 years), of whom 29 (72%) had Eisenmenger syndrome and 14 (35%) had Down syndrome. The number of 1MSTST repetitions correlated significantly with 6MWT distance (r = 0.807, p = 0.000). There were no adverse events, and the 1MSTST results correlated with the WHO functional class. Heart rate increase and oxygen desaturation after both tests correlated significantly, but less desaturation was observed after 1MSTST. CONCLUSIONS: Our study showed that the 1MSTST is a safe and easily applicable test in adult patients with PAH-CHD, including patients with Down syndrome. The results of the 1MSTST correlate significantly with the 6MWT, providing an alternative tool for exercise capacity assessment in patients with PAH-CHD.

Thirty years of surgical management of pediatric pulmonary hypertension: Mid-term outcomes following reverse Potts shunt and transplantation.

BACKGROUND: Reverse Potts shunt (RPS) and lung or heart-lung transplantation are life-extending surgical interventions for pediatric patients with severe pulmonary arterial hypertension (PAH). Robust criteria for identifying patients who will benefit from these procedures remain elusive. Based on 30 years of experience, we sought to refine the surgical indications. METHODS: This single-center retrospective cohort study included 61 consecutive pediatric patients with PAH managed by RPS (2004-2020) or transplantation (1988-2020). Their mid-term outcomes were assessed. RESULTS: Compared with the 20 patients managed by RPS, the 41 transplant waitlist patients, of whom 28 were transplanted, were older (14.9 vs 8.0 years, P = .0001), had worse right ventricular impairment (tricuspid annular plane systolic excursion, 12.5 mm vs 18.0 mm, P = .03), and were managed later in the evolution of the disease (6.0 vs 1.7 years, P = .002). After implementation of a high-priority allocation program in 2007, waitlist mortality decreased from 52.6% to 13.6% (P = .02) and 5-year survival increased from 57.1% to 74.7% after RPS and 55.6% to 77.2% after transplantation. At a median follow-up of 8.6 years after RPS and 5.9 years after transplantation, functional capacity had improved significantly, and PAH-specific drug requirements had diminished markedly in the RPS group. Two patients successfully underwent double-lung transplant 6 and 9 years after RPS. CONCLUSIONS: In selected children with suprasystemic PAH, RPS is associated with functional capacity improvements and decreased pharmacotherapy needs over the midterm. RPS deserves consideration earlier in the course of pediatric PAH, with transplantation being performed in the event of refractory RV failure.

A case of atrial septal defect with right-to-left shunting without pulmonary hypertension.

We report a case of a 54-year-old man with atrial septal defect who presented with oxygen desaturation on pulse oximetry. Cardiac magnetic resonance imaging and transesophageal echocardiography showed right-to-left shunting through an atrial septal defect, which was confirmed by superior vena cavography and suggested Eisenmenger syndrome. However, cardiac catheterization revealed a normal pulmonary arterial pressure. Simultaneous measurement of interatrial pressure identified two transient interatrial pressure gradient points, where the right atrial pressure was higher than the left atrial pressure. The patient was finally diagnosed with atrial septal defect without pulmonary hypertension. Right-to-left shunting was primarily caused by a transient interatrial pressure gradient due to a time delay in both initial atrial contraction and completion of passive ventricular filling between the right and left sides of the heart. Surgical closure of the atrial septal defect was performed, and hypoxemia improved. This is the first report of right-to-left shunting without pulmonary hypertension caused by a transient interatrial pressure gradient due to a time delay between the right and left cardiac cycles. Precise assessment of the simultaneous interatrial pressure in addition to diagnostic imaging played a pivotal role in clarifying the etiology of this rare condition. Learning objective: Atrial septal defect with right-to-left shunting without Eisenmenger syndrome is a rare condition. We identified transient interatrial pressure gradients associated with a time delay in both initial atrial contraction and completion of the passive ventricular filling phase, which we considered as the primary mechanism underpinning right-to-left shunting. Simultaneous measurement of interatrial pressure played a pivotal role in elucidating the hemodynamics and abnormal shunt flow mechanism.

Anesthetic management of a pregnant patient with Eisenmenger's syndrome: A case report.

BACKGROUND: Eisenmenger's syndrome (ES) is a rare complication of congenital heart disease that includes pulmonary artery hypertension and reversed or bidirectional shunts. The mortality rate of pregnant women with ES is 30%-70% due to pathophysiological deterioration. Successful perioperative management of a pregnant patient with ES is a challenge for anesthesiologists. CASE SUMMARY: A 38-year-old pregnant woman was admitted to the cardiology department of our hospital at 22 wk of gestation with complaints of chest tightness and shortness of breath for 3 wk. Transthoracic echocardiography revealed a bidirectional shunt between the descending aorta and pulmonary artery after interventional closure of the patent ductus arteriosus and severe pulmonary hypertension. ES in pregnancy was our primary suspicion. The patient elected to terminate the pregnancy under adequate preoperative preparation, rigorous intraoperative monitoring, and perfect epidural anesthesia. She was discharged successfully on postoperative day 16. CONCLUSION: Our experience in this case suggests that successful outcomes are possible in pregnant patients with ES for termination of pregnancy under epidural anesthesia and intensive monitoring.

Clinical features and pregnancy outcomes in women with aortopulmonary window defect: case series.

Background: Aortopulmonary window is a rare congenital heart defect that results in severe pulmonary arterial hypertension (PAH), Eisenmenger syndrome, and congestive heart failure in the first months of life. Pregnancy is absolutely contraindicated in the patients with this condition. Case summary: This paper describes two clinical cases of pregnancy in patients (28 and 20 years old) with aortopulmonary window defect, severe PAH, and Eisenmenger syndrome that ended in preterm delivery by caesarean section. One patient died in the postpartum period due to progression of right ventricular heart failure. The younger patient survived childbirth and the postpartum period; later, she continued therapy at the PAH centre. Discussion: We describe unusual cases of clinical features in pregnant women with aortopulmonary window defect. Due to the rare occurrence and low survival rate of patients with uncorrected aortopulmonary window defect, descriptions of clinical cases of this defect in adults are very rare. It is very important to note the necessity of observation of these patients in specialized centres by a multidisciplinary team of healthcare professionals, due to the high risk of cardiovascular, obstetric complications, and death.