Diagnostic and Management Considerations in a High-Risk Pregnant Patient With Ischemic Cardiomyopathy.

Ischemic heart disease is an important cause of heart failure in pregnancy. Involvement of a cardio-obstetrics team is crucial for managing high-risk pregnant patients with cardiovascular disease. We present a case of cardiogenic shock in a pregnant woman unmasking underlying multivessel obstructive coronary artery disease.

Sudden death in adults with repaired coarctation of the aorta: A case for sex-based risk factors.

Background: Sudden cardiac death (SCD) is an important risk for adults with repaired coarctation of the aorta (rCoA). We aimed determine if there are clinical risk factors for SCD in adults with rCoA. Methods and results: SCD events and clinical data from all adults with rCoA at a tertiary care center (2007-2017) were evaluated. In 167 adults with rCoA (39 ± 11 years old, 75 (45%) female) SCD occurred in 8 (5%) (vs. age-matched adults 0.9%). Those with SCD demonstrated significant QTc prolongation (QTc: 479 ± 16 vs. 434 ± 30 msec, p < 0.001). Overall, adults with rCoA and a prolonged sex-normative QTc interval had a 12-fold increased risk of SCD (x2 (1) = 12.3, p < 0.001), with men sustaining SCD at younger ages (42 ± 13 years vs. women 60 ± 10 years, p < 0.05). Multiple logistic regression modeling demonstrated that prolonged QTc selectively advanced risk for SCD in men only (x2 QTc prolongation 8.46, p < 0.005 and x2 age 0.29, p = 0.587), whereas in women, age was associated with SCD risk (x2 QTc prolongation 2.84, p = 0.092 and x2 age 7.81, p = 0.005). Non-sustained ventricular tachycardia, ventricular dysfunction, and myocardial fibrosis did not significantly impact SCD risk. Conclusions: There is an unanticipated high burden of SCD in adults with rCoA, occurring in men at younger age than women, suspicious for primary electrophysiologic dysfunction. Future investigation of sex-specific SCD risk in rCoA is important to better understand this disease and its late phenotype.

Novel uses for implanted haemodynamic monitoring in adults with subaortic right ventricles.

BACKGROUND: Pulmonary hypertension (PH) is a common complication in patients with complete dextro-transposition of the great arteries (TGA) after atrial switch (D-TGA/AS) and congenitally corrected TGA (ccTGA). In this population with subaortic right ventricles (sRVs), echocardiography is a poor screening tool for PH; implantable invasive haemodynamic monitoring (IHM) could be used for this purpose, but data are limited. The aim of this study is to report on novel uses of IHM in patients with sRV. METHODS: This retrospective study describes the uses of IHM, impact of IHM on heart failure hospitalisation (HFH) and device-related complications in adults with sRV from a single centre (2015-2022). RESULTS: IHM was placed in 18 patients with sRV (median age 43 (range 30-54) years, 8 female, 16 with D-TGA/AS, 2 with ccTGA); 16 had moderate or severe sRV systolic dysfunction, 13 had PH on catheterisation. IHM was used for (1) Medical therapy titration, (2) Medical management after ventricular assist device in patients with transplant-limiting PH and (3) Serial monitoring of pulmonary artery pressures without repeat catheterisations to help identify the optimal time for heart transplant referral. In follow-up (median 23 months), HFHs/year were similar to the year prior to IHM (median 0 (IQR 0-1.0) before vs 0 (0-0.8) after, p=0.984). Device migration occurred in one, without long-term sequelae. CONCLUSIONS: Uses of IHM in patients with sRV are described which may minimise the need for serial catheterisations in a population where PH is prevalent. HFHs were low overall but not impacted by IHM. One device-related complication occurred without long-term consequence.

Double-Chambered Right Ventricle Presenting in Adulthood With Aortic and Pulmonic Valve Endocarditis.

A 55-year-old woman admitted with symptoms of right heart failure received a diagnosis of double-chambered right ventricle and a ventricular septal defect with aortic and pulmonic valve endocarditis. This case highlights the use of multimodality imaging and importance of adult congenital heart disease expertise in the diagnosis and treatment of such patients. (Level of Difficulty: Intermediate.).

Maternal and fetal outcomes in pregnant women with pulmonary hypertension: The impact of left heart disease.

Background: Pulmonary hypertension (PH) due to left heart disease (World Health Organization (WHO) Group 2 PH) is the largest PH subgroup, however most reports of PH in pregnancy focus on patients with pulmonary arterial hypertension (WHO Group 1 PH). We evaluated pregnancy outcomes across WHO PH subgroups. Methods: We performed a retrospective single center cohort study of maternal and fetal outcomes in pregnant women with PH (2004-2018). Results: We analyzed outcomes of 70 pregnancies in 70 women with PH (30 ± 6 years-old), classified as WHO Group 1 PH (12 (17%)), Group 2 PH (45 (64%)), Group 3 PH (4 (6%)) and Group 5 PH (9 (13%)). Although no peripartum death occurred, 3 (4.3%) women with WHO Group 2 PH had late mortality (7 ± 4 months post- partum). Additionally, 33 major adverse cardiac events occurred in 26 (37%) women, preterm birth occurred in 32 (49%), and post-partum hemorrhage in 10 (14%), such that only 24 (37%) women completed a viable pregnancy free of an adverse cardiac, obstetric or fetal/neonatal event. Major adverse cardiac events were predominantly due to heart failure (24 (73%)), occurring only in WHO Groups 1 and 2 PH (3 (25%) women vs. 17 (38%), p = 0.07), and significantly associated with pre-eclampsia, left ventricular ejection fraction ≤45%, maternal diabetes, and systemic hypertension. Conclusions: WHO Group 2 PH carries similar risk for maternal cardiovascular events when compared to women with WHO Group 1 PH. Further studies evaluating maternal risk in this cohort are needed.

Pulmonary vasodilators: beyond the bounds of pulmonary arterial hypertension therapy in COVID-19.

Pulmonary arterial hypertension (PAH) and novel coronavirus (SARS-CoV-2) disease COVID-19 are characterized by extensive endothelial dysfunction and inflammation leading to vascular remodeling and severe microthrombi and microvascular obliterative disease. It is hypothesized that those patients with underlying lung disease, like PAH, represent a high-risk cohort in this pandemic. However, reports of COVID-19 in this cohort of patient have been scaring and an observational survey showed that the disease was relatively well tolerated. We postulate that specific PAH vasodilator may offer some protection and/or advantage in the case of concomitant COVID-19. Here we review the literature describing mechanisms of action for each of the broad categories of PAH therapy, and offer potential hypothesis about why this therapy may impact outcomes in COVID-19.

Triage and management of the ACHD patient with COVID-19: A single center approach.

With the recent emergence of SARS-CoV-2 and COVID-19, healthcare facilities and personnel are expected to rapidly triage and care for patients with even the most complex medical conditions. Adults with congenital heart disease (ACHD) represent an often-intimidating group of complex cardiovascular disorders. Given that general internists and general cardiologists will often be asked to evaluate this group during the pandemic, we propose here an abbreviated triage algorithm that will assist in identifying the patient's overarching ACHD phenotype and baseline cardiac status. The strategy outlined allows for rapid triage and groups various anatomic CHD variants into overarching phenotypes, permitting care teams to quickly review key points in the management of moderate to severely complex ACHD patients.

The adult with a fontan: a panacea without a cure? Review of long-term complications.

The univentricular heart includes a spectrum of complex cardiac defects that are managed by staged palliative surgical procedures, ultimately resulting in a Fontan procedure. Since 1971, when it was first developed, the procedure has undergone several variations. These patients require lifelong management, including a thorough knowledge of their anatomic substrate, hemodynamic status, management of rhythm and ventricular function, together with multi-organ evaluation. As these patients enter middle age, there is increasing awareness of long-term complications and mortality. This review highlights the concept behind the staged surgical palliations, the unique single ventricle physiology and the long-term complications in this complex cohort of patients.

A phase II tolerability study of cisplatin plus docetaxel as adjuvant chemotherapy for resected non-small cell lung cancer.

INTRODUCTION: We undertook this phase II study to measure postoperative drug delivery and toxicity of cisplatin plus docetaxel in patients with resected stage I-III non-small cell lung cancer. METHODS: The primary endpoint was amount of cisplatin delivered over a planned four cycles of adjuvant chemotherapy. Statistical design required a cohort to close if the regimen proved unlikely to improve cisplatin delivery compared with published phase III data. The first cohort was treated with docetaxel 35 mg/m2 intravenously (IV) on days 1, 8, and 15, and cisplatin 80 mg/m2 IV on day 15, every 4 weeks for four planned cycles. A second cohort was treated with docetaxel 75 mg/m2 IV plus cisplatin 80 mg/m2 IV on day 1 every 3 weeks for four planned cycles. RESULTS: Sixteen patients were treated with weekly docetaxel and cisplatin every 4 weeks, with five of 16 (31%) unable to complete three cycles. Subsequently, 11 patients were treated with docetaxel and cisplatin every 3 weeks, with six of 11 (55%) unable to complete three cycles. Among the 11 patients who failed to complete three cycles, the reasons for stopping included one or more of the following: fatigue (n = 8), nausea (n = 4), febrile neutropenia (n = 1), hypotension (n = 1), and nephrotoxicity (n = 1). CONCLUSIONS: The combination of cisplatin at 80 mg/m2 with docetaxel 35 mg/m2 weekly or 75 mg/m2 every 3 weeks is no better tolerated than older chemotherapy regimens. The most common reason to stop chemotherapy was intolerable fatigue. These results suggest that the most common dose-limiting toxicities are attributable to the cisplatin, given similar problems were encountered whether the docetaxel was delivered as a single dose every 3 weeks or as a lower weekly dose.