Current Practice in Carcinoid Heart Disease and Burgeoning Opportunities.

OPINION STATEMENT: Cardiac surgery with tricuspid valve and potentially pulmonic valve replacement at an experienced center is currently the most effective strategy available for the treatment of carcinoid heart disease. Cardiac surgery for carcinoid heart disease requires a multidisciplinary team including cardiology, medical oncology, cardiothoracic anesthesia, and cardiac surgery. Without cardiac surgery, morbidity and mortality from carcinoid heart disease is high. Aggressive management of carcinoid before and after cardiac surgery is critical. Over time, though, circulating carcinoid hormones can lead to destruction of prosthetic valves as well, resulting in recurrent right heart failure. Percutaneous options for valve repair may be on the horizon for management of carcinoid heart disease.

Bilateral lung transplantation for pulmonary artery aneurysm with severe pulmonary hypertension: An evolution or a revolution?

The surgical treatment of pulmonary hypertension (PH), with or without pulmonary artery aneurysm, has evolved during the last 40 years from heart-lung transplants to bilateral lung transplants as the treatment of choice for PH patients with preserved right and left ventricular function and without complex cardiac abnomalies.

Tricuspid and Pulmonic Valve Pathology.

PURPOSE OF REVIEW: This review describes the normal structure and pathologic changes that affect the right-sided cardiac valves and chambers. RECENT FINDINGS: The anatomy and pathology described have been known for many years. Knowledge of these findings has gained relevance. The pattern of endocarditis is changing. New diagnostic techniques have allowed better characterization of lesions responsible for cardiac dysfunction. Novel, less invasive interventions have made recognition of abnormalities more clinically relevant. There are many different pathologic entities that can affect the right-sided cardiac valves. These are discussed in this review.

Pulmonic Valve Disease: Review of Pathology and Current Treatment Options.

PURPOSE OF REVIEW: Our review is intended to provide readers with an overview of disease processes involving the pulmonic valve, highlighting recent outcome studies and guideline-based recommendations; with focus on the two most common interventions for treating pulmonic valve disease, balloon pulmonary valvuloplasty and pulmonic valve replacement. RECENT FINDINGS: The main long-term sequelae of balloon pulmonary valvuloplasty, the gold standard treatment for pulmonic stenosis, remain pulmonic regurgitation and valvular restenosis. The balloon:annulus ratio is a major contributor to both, with high ratios resulting in greater degrees of regurgitation, and small ratios increasing risk for restenosis. Recent studies suggest that a ratio of approximately 1.2 may provide the most optimal results. Pulmonic valve replacement is currently the procedure of choice for patients with severe pulmonic regurgitation and hemodynamic sequelae or symptoms, yet it remains uncertain how it impacts long-term survival. Transcatheter pulmonic valve replacement is a rapidly evolving field and recent outcome studies suggest short and mid-term results at least equivalent to surgery. The Melody valve® was FDA approved for failing pulmonary surgical conduits in 2010 and for failing bioprosthetic surgical pulmonic valves in 2017 and has been extensively studied, whereas the Sapien XT valve®, offering larger diameters, was approved for failing pulmonary conduits in 2016 and has been less extensively studied. Patients with pulmonic valve disease deserve lifelong surveillance for complications. Transcatheter pulmonic valve replacement is a novel and attractive therapeutic option, but is currently only FDA approved for patients with failing pulmonary conduits or dysfunctional surgical bioprosthetic valves. New advances will undoubtedly increase the utilization of this rapidly expanding technology.

Pulmonic valve fibroelastoma-A rare incidental finding.

Primary cardiac neoplasms are a rare, commonly benign, tumor with an approximate incidence rate of 0.02%. Papillary fibroelastoma (PFE), a common form of primary cardiac neoplasms, typically present as a mass on the aortic and mitral valves, while rarely presenting as a pulmonary valve tumor. The majority of PFEs are asymptomatic, however valvular masses can pose a significant health hazard due to their potential to fragment into the bloodstream, facilitate thrombus formation, and restrict blood flow. Due to these risks, careful resection of the mass is recommended for symptomatic patients and asymptomatic patients if the tumor is large (>1 cm), mobile, or on left-sided valves. Here we present a case of an incidental finding of a pulmonic valve papillary fibroelastoma in a 65-year-old man by transesophageal echocardiography during a coronary artery bypass graft procedure.

Streptococcus oralis pulmonic valve endocarditis: a case report and review of the literature.

BACKGROUND: Several factors increase the risk of right-sided endocarditis. The tricuspid valve is usually involved in right-sided endocarditis cases. Infective endocarditis of the pulmonic valve is rare, and few cases of pulmonic valve endocarditis were reported previously. CASE PRESENTATION: Here we describe a case of a 81-year-old Middle Eastern male patient, admitted to our hospital three times in a period of 2 months for fever and cough. He had Streptococcus oralis bacteremia with vegetation that was on the pulmonic valve. We diagnosed him with pulmonic valve endocarditis, and he was treated successfully with intravenous antibiotics. CONCLUSION: It is important to keep high suspicion for isolated pulmonic valve endocarditis in patients with respiratory symptoms. Adequate dental care is important in patients with risk factors for infective endocarditis.

Retrieval of Large Balloon Fragments During Transcatheter Pulmonary Valve Implantation Using a Novel Retrieval System.

The removal of balloon fragments from the pulmonary artery without damaging the pulmonary and tricuspid valves can be difficult. Four cases during transcatheter pulmonary valve replacement are described in which a novel retrieval system was used to facilitate safe removal. (Level of Difficulty: Advanced.).

Possible Isolated Pulmonic Valve Endocarditis in a Patient With Sickle Cell Disease: A Case Report.

Pulmonic valve endocarditis is a rare and clinically elusive identity, commonly associated with congenital heart malformations and intravenous (IV) drug abuse. We describe a case of a 40-year-old male who has established sickle cell disease and presented with pain crisis, febrile episodes, and oxygen desaturation on room air. The clinical presentation and echocardiographic findings of a pulmonic mass were consistent with the diagnosis of pulmonic valve endocarditis. Due to the small size of the pulmonic valve vegetation, the patient was treated with antibiotics and discharged home on antibiotics and home oxygen.

Left Atrial Hypertension and Respiratory Failure Requiring Venoarterial ECMO After Transcatheter Pulmonary Valve Replacement.

A 33-year-old woman with aortic valve stenosis status-post Ross at age 6 years developed symptomatic right heart failure from right ventricle to pulmonary artery conduit stenosis. Conduit rehabilitation and transcatheter pulmonary valve replacement resulted in acute left atrial hypertension and respiratory failure requiring venoarterial extracorporeal membrane oxygenation and atrial septal defect creation as a bridge to recovery.

Self-Expanding Transcatheter Pulmonary Valve Implant in the Right Pulmonary Artery.

Newer self-expanding transcatheter pulmonary valves (TPVs) are approved for the treatment of severe pulmonary regurgitation in patients with large right ventricular outflow tracts. We present a patient with Tetralogy of Fallot whose right ventricular outflow tract was too large for self-expanding TPV, who was treated successfully with a self-expanding TPV in the right pulmonary artery. (Level of Difficulty: Advanced.).

Balancing carcinoid crisis and right ventricular dysfunction during tricuspid and pulmonic valve replacement for carcinoid heart disease: A case report.

INTRODUCTION AND IMPORTANCE: Carcinoid tumors are rare malignancies of neuroendocrine origin that can manifest with a constellation of systemic symptoms including right-sided cardiac involvement. Many patients with carcinoid heart disease require valve replacement, but intraoperative management of carcinoid syndrome varies within the literature. CASE PRESENTATION: A 72-year-old man with carcinoid syndrome underwent tricuspid and pulmonic valve replacement with multiple episodes of carcinoid crisis intraoperatively as well as right ventricular dysfunction after cardiopulmonary bypass. CLINICAL DISCUSSION: Octreotide is the mainstay in prevention and treatment of intraoperative carcinoid crisis, but reported dosages and timing varies significantly. The use of exogenous catecholamines is also controversial as they are thought to paradoxically worsen carcinoid symptoms. Our patient was managed successfully with both an octreotide infusion and intermittent boluses, as well as exogenous catecholamines for right ventricular support during and after cardiopulmonary bypass. CONCLUSION: The management of carcinoid syndrome in patients undergoing valve surgery for carcinoid heart disease is dependent on timely prevention and treatment of carcinoid crisis and effective mitigation of right ventricular dysfunction.

Left Pulmonary Artery Aneurysm: A Post-stenotic Pulmonary Aneurysm Related to Pulmonary Valve Stenosis.

Aneurysms of the pulmonary artery are uncommon vascular pathologies that are associated with congenital structural cardiac anomalies, pulmonary hypertension, vasculitis, neoplasm, iatrogenic, and infection. PAAs are commonly asymptomatic and accidentally diagnosed, however, if symptomatic, clinical features are generally non-specific and depend on the etiology of PAA. CT pulmonary angiography remains the gold standard imaging modality and other diagnostic imaging tests include transthoracic echocardiography and right heart catheterization. Definitive treatment of PAA is surgery, however, conservative management with close monitoring should be practiced in patients with poor surgical candidates or surgery is unlikely to improve survival. Here, we report a case of pulmonary artery aneurysm secondary to congenital pulmonary valve stenosis as well as a brief review of the literature regarding pulmonary artery aneurysms.

Asymptomatic Pulmonic Valve Mass: A Diagnostic and Therapeutic Dilemma.

Cardiac masses are not common but remain important in cardiology practice as they can cause havoc to a patient's life through obstructive and arrhythmogenic symptoms. These lesions mostly include vegetation, thrombi, and tumors. Myxomas are the most common primary cardiac tumor, primarily arising from the left heart chambers. It is exceedingly rare for a myxoma to emerge from the right-sided cardiac valves. The standard treatment is surgical resection, regardless of size, which is not always possible. We report a unique case of a male with multiple co-morbidities who presented with an incidental finding of a pulmonary valve mass suspicious of being a myxoma. The myxomatous mass was asymptomatic with no right ventricular outflow tract obstruction. Echocardiogram can help identify and characterize these lesions, but this may not be easy, especially in the case of atypical location or morphology of the mass. Similarly, in some cases, the patient may not be able to undergo surgical excision. In such cases, there is no consensus or guidelines to help clinicians best manage the patients medically, creating a diagnostic and therapeutic dilemma.

Isolated Pulmonic Valve Endocarditis.

Isolated pulmonic valve infective endocarditis (PV-IE) is a rare form of endocarditis. The authors report a case of giant vegetations detected by transthoracic echocardiography (TTE) on PV in a young patient, 33-year-old, with drug abuse history. The patient underwent surgical intervention by pulmonary valved bioconduit implantation. After operation, a pulmonary embolism episode was treated by a direct oral anticoagulant. The final outcome was favorable. The difficulty in diagnosing PV-IE is due to the inability to properly visualize the PV by echocardiography. In this case, with such large vegetations, TTE allowed a correct diagnosis and an effective surgical planning, confirming its importance as a diagnostic tool.

Risk factors and long-term outcomes after tetralogy of Fallot repair at an Asian tertiary referral center.

BACKGROUND: Tetralogy of Fallot is the most common type of cyanotic congenital heart disease. More postoperative tetralogy of Fallot patients grow up than in the past, and these patients need to be followed-up. OBJECTIVE: To investigate the survival and long-term outcomes of patients who underwent total repair of tetralogy of Fallot, and to identify the risk factors for reoperation with pulmonic valve replacement. METHOD: A total of 403 patients who underwent total tetralogy of Fallot repair at our center during 1997 to 2016 were retrospectively included. Demographic, clinical, treatment, outcome, and follow-up data were collected and analyzed. RESULTS: Median age and body weight at the time of tetralogy of Fallot repair was 4.41 years (range: 0.85-55.28) and 13.58 kg (range: 5.5-68), respectively. The median follow-up was 9.0 years, and overall mortality was 3.2%. The actuarial survival rates at 10 and 20 years were 96.4% and 95.2%, respectively, and the freedom from pulmonic valve replacement was 93.4% and 57.4%, respectively. The median time to indicate pulmonic valve replacement was 13.9 years (range: 6.2-20.5). Multivariate analysis revealed transannular patch technique (hazard ratio: 3.023, 95% confidence interval: 1.34-6.83; p = 0.008) and palliative shunt (hazard ratio: 2.39, 95% confidence interval: 1.16-4.91; p = 0.018) to be independent risk factors for reoperation with pulmonic valve replacement. CONCLUSION: The rates of overall survival and freedom from pulmonic valve replacement were both high in this study, and both were comparable to the rates reported from other studies. Overall mortality was as low as 3.47%. The need for a transannular patch or palliative shunt should be considered risk factors for a consequent reoperation.

Simultaneous Tricuspid and Pulmonic Valve Replacement Due to Infective Endocarditis.

Right-sided valvular infective endocarditis (RSIE) is often associated with intravenous (IV) drug abuse and typically involves the tricuspid valve. The involvement of both the tricuspid and pulmonic valves is a rare entity. A 39-year-old woman presented with fever, dyspnea on exertion, and chest pain. She was subsequently found to have infectious endocarditis (IE) with the involvement of both the tricuspid and pulmonic valves. Simultaneous tricuspid and pulmonic valvular repair with bioprosthetic valves were performed with bovine pericardium to reconstruct the anterior surface of the pulmonary artery. Recovery was complicated by the development of a complete atrioventricular (AV) block requiring pacemaker implantation. Following device placement, the patient also developed two episodes of ventricular tachycardia arrest likely precipitated by the device. Return of spontaneous circulation (ROSC) was achieved and no further episodes occurred once the device was exchanged with a cardiac resynchronization therapy defibrillator. The patient improved clinically and was discharged home with no further complications.

A Rare Case of Pulmonic and Aortic Valve Infective Endocarditis: A Case Report.

Infective endocarditis (IE) is a microbial infection affecting cardiac valves. IE most often affects the aortic valve and is commonly caused by community-acquired, penicillin-sensitive streptococcus that enters through the oral cavity. In this report, we present a case of a 66-year-old man with a medical history of congenital pulmonic stenosis status after pulmonic valve (PV) repair. The patient underwent a transesophageal echocardiogram showing a 1 cm × 0.7 cm mobile vegetation attached to the ventricular aspect of the right coronary aortic cusp and a 1.1 cm × 0.5 cm mobile vegetation attached to the arterial aspect of the PV cusp. In conclusion, concomitant right- and left-sided IE is an exceedingly rare condition. Due to its rarity and complexity of presentation, pulmonic valve endocarditis (PVE) requires a multidisciplinary approach to its perioperative management to prevent systemic complications.

Active infective endocarditis of both aortic and pulmonic valves in association with ventricular septal defect treated with double valve replacement and closure of the defect.

Described herein is a 29-year-old man with a ventricular septal defect who developed active infective endocarditis on both his pulmonic and aortic valves. We found only six previously reported cases partially similar to ours.