Ventricular Fibrillation Arrest and Pulmonary Homograft Rupture During Transcatheter Pulmonary Valve: Successful Rescue by Heart Team

We describe a 31-year-old woman with pulmonary homograft rupture and ventricular fibrillation arrest complicating a transcatheter pulmonary valve (TPV) procedure. She underwent extracorporeal membrane oxygenation (ECMO) with immediate surgical repair including bioprosthetic pulmonary valve replacement. She had difficulty weaning off ECMO due to hyperacute failure of the valve and ultimately underwent successful hybrid TPV with complete recovery. This case illustrates the importance of the heart team approach during catheter and surgical interventions in adult congenital heart disease.

Evaluation and Management of Pulmonary Arterial Hypertension in Congenital Heart Disease

Pulmonary arterial hypertension is a common complication in patients with congenital heart disease (CHD), aggravating the natural course of the underlying defect. Pulmonary arterial hypertension (PAH) has a multifactorial etiology depending on the size and nature of the cardiac defect as well as environmental factors. Although progress has been made in disease-targeting therapy using pulmonary vasodilators to treat Eisenmenger syndrome, important gaps still exist in the evaluation and management of adult patients with CHD-associated PAH (PAH-CHD) who have systemic-to-pulmonary shunts. The choice of interventional, medical, or both types of therapy is an ongoing dilemma that requires further data. This review focuses on the evaluation and management of PAH-CHD in the contemporary era.

Chronic Thromboembolic Pulmonary Hypertension Medical Management

Chronic thromboembolic pulmonary hypertension (CTEPH) is a common long-term complication of pulmonary embolism characterized by thromboembolic obstruction of the pulmonary arteries, vascular arteriopathy, vascular remodeling, and ultimately pulmonary hypertension (PH). Although pulmonary endarterectomy (PEA) surgery is the standard of care, approximately 40% of patients in the international CTEPH registry were deemed inoperable. In addition to lifelong anticoagulation, the cornerstone of PH-specific medical management is riociguat, a soluble guanylate cyclase stimulator. Medical management should be started early in CTEPH patients and may be used as a bridge to PEA surgery or balloon pulmonary angiography. Medical management is indicated for inoperable CTEPH patients and patients who have recurrence of PH after PEA surgery.

Novel Treatment Pathways in Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a severe and progressive vascular disease characterized by pulmonary vascular remodeling, proliferation, and inflammation. Despite the availability of effective treatments, PAH may culminate in right ventricular failure and death. Currently approved medications act through three well-characterized pathways: the nitric oxide, endothelin, and prostacyclin pathways. Ongoing research efforts continue to expand our understanding of the molecular pathogenesis of this complex and multifactorial disease. Based on recent discoveries in the pathobiology of PAH, several new treatments are being developed and tested with the goal of modifying the disease process and ultimately improving the long-term prognosis.

Percutaneous Thrombus Removal in COVID-19–Infected Patient with Pulmonary Embolism

We present a case describing the use of the AngioVac system (AngioDynamics, Inc.) and SENTINEL™ cerebral protection system (SCPS; Boston Scientific) in a patient with COVID-19 who initially presented with a large deep-vein thrombosis of the left lower extremity, complicated by a pulmonary embolism. Although he initially improved with systemic alteplase, he later developed a second large clot diagnosed in transit in the right atrium. Within 12 hours from initial thrombolysis, this large clot wedged across an incidental patent foramen ovale (PFO), the atrial septum, and the cavotricuspid annulus. We emergently performed a percutaneous clot extraction with preemptive placement of the SCPS in anticipation of cardioembolic phenomenon. A large (> 10 cm) clot was extracted without complication, and the patient was discharged home. The combined use of SCPS and AngioVac in this case suggests a potential role for percutaneous treatment of severe and consequential thromboembolic disease, especially in patients with a PFO, and may be considered as an alternative and less-invasive option in patients with COVID-19. While cerebral embolic protection devices are approved for and widely used in transcatheter aortic valve replacement procedures, there is a theoretical benefit for use in percutaneous thrombolectomies as well.

Evaluation, Diagnosis, and Classification of Pulmonary Hypertension

Pulmonary hypertension (PH) is a rare heterogenous disease characterized by elevated blood pressure in the lungs. Patients with PH require careful evaluation and management at an expert center. Understanding of the mechanisms underlying the development of PH has increased over the past two decades, and several treatment options for pulmonary arterial hypertension have emerged. Despite this progress, PH continues to carry high morbidity and mortality. The 6th World Symposium on Pulmonary Hypertension that occurred in late 2018 modified the clinical classification of PH into five groups. In this review, we focus on the evaluation and diagnosis of PH and discuss the updated clinical classification.

Bradycardia in Recent Heart Transplant: Will the Microscope Illuminate the True Answer?

Transplant recipients are at risk of developing rejection that may cause significant morbidity and mortality following transplantation. The clinical presentation of rejection may be atypical, leading to difficulties in diagnosis and management especially in cases with a nondiagnostic biopsy specimen. The emergence of artificial intelligence may aid in clinical decision making when traditional techniques are inconclusive.

Management of Pulmonary Hypertension in Left Heart Disease

Pulmonary hypertension due to left heart diseases (PH-LHD) is the most prevalent form of pulmonary hypertension. It frequently complicates heart failure with reduced ejection fraction (HFrEF) or preserved ejection fraction (HFpEF) and negatively impacts prognosis, particularly when a precapillary component is present. PH-LHD is distinctive from pulmonary arterial hypertension (PAH) even though both conditions may share some common characteristics. In addition, the mechanisms involved in the development of a precapillary component are yet to be fully clarified, in particular in PH due to HFpEF. Several studies have been exploring PAH pathways as potential therapies for PH-LHD, but no PAH-approved drug has demonstrated efficacy in PH-LHD. Rather, some classes of drugs, such as endothelin-receptor antagonists or prostacycline-analogues, have been found to be harmful in patients with HF. Therefore, at present, the only established treatments for PH-LHD are those that target the heart as recommended in the international guidelines for HF. Based on current knowledge, off-label prescription of PAH-approved drugs in PH-LHD patients must be strongly discouraged.