Ventricular Inflow Obstruction Research Articles

Perioperative and Anesthetic Considerations in Shone's Complex.

Shone's complex is a congenital cardiac disease consisting of the following four lesions: parachute mitral valve, supravalvar mitral ring, subaortic stenosis, and aortic coarctation. Though not all components are required for a diagnosis, the end result is both left ventricular inflow and outflow obstruction, which typically present in patients as congestive heart failure. The complex pathology requires careful management and surgical decision-making to ensure an optimal outcome. This review will focus on the anatomy, physiology, and perioperative anesthetic management of patients with Shone's complex.

Surgical Considerations in Shone Complex.

Shone complex is defined by 4 anomalies: parachute mitral valve, supravalvar mitral ring, subaortic stenosis, and coarctation of the aorta. Establishing a clear definition is one of the principal challenges in the study of Shone complex as not all patients have all lesions. The essential feature of Shone complex is multilevel left-sided obstruction involving both the left ventricular inflow and outflow. This anatomic variability is reflected in the clinical presentation as signs of left ventricular inflow obstruction are often masked by outflow obstruction and the multilevel nature of the condition is thus underappreciated. Surgical treatment is often stepwise addressing the outflow obstruction first. In this review, geared to the pediatric cardiac anesthesiologist, we review the pathophysiology, diagnosis, treatment, and outcomes of Shone complex.

Decreased left heart flow in fetal lambs causes left heart hypoplasia and pro-fibrotic tissue remodeling

Low blood flow through the fetal left heart is often conjectured as an etiology for hypoplastic left heart syndrome (HLHS). To investigate if a decrease in left heart flow results in growth failure, we generate left ventricular inflow obstruction (LVIO) in mid-gestation fetal lambs by implanting coils in their left atrium using an ultrasound-guided percutaneous technique. Significant LVIO recapitulates important clinical features of HLHS: decreased antegrade aortic valve flow, compensatory retrograde perfusion of the brain and ascending aorta (AAo) from the arterial duct, severe left heart hypoplasia, a non-apex forming LV, and a thickened endocardial layer. The hypoplastic AAo have miRNA-gene pairs annotating to cell proliferation that are inversely differentially expressed by bulk RNA-seq. Single-nucleus RNA-seq of the hypoplastic LV myocardium shows an increase in fibroblasts with a reciprocal decrease in cardiomyocyte nuclei proportions. Fibroblasts, cardiomyocytes and endothelial cells from hypoplastic myocardium have increased expression of extracellular matrix component or fibrosis genes with dysregulated fibroblast growth factor signaling. Hence, a severe sustained ( ~ 1/3 gestation) reduction in fetal left heart flow is sufficient to cause left heart hypoplasia. This is accompanied by changes in cellular composition and gene expression consistent with a pro-fibrotic environment and aberrant induction of mesenchymal programs.

Cardiac myxomas: characteristics and outcomes in a tertiary center

Abstract Introduction Cardiac myxoma (CM) is the most common primary cardiac tumour. It can manifest in a variety of clinical presentations or it can be asymptomatic and, ultimately, be detected with image techniques. The only successful treatment of these tumours is surgical excision, with low complications and recurrence rate. Objectives This analysis aims to describe the clinical forms of presentation and echocardiographic characteristics of cardiac myxomas, establish myxoma features associated with obstructive symptons, as well as describe the postoperative outcome and long-term recurrence in a tertiary center. Methods Between 1990 and 2021, 88 patients were diagnosed with cardiac myxoma. 84 were included in this analysis. Baseline characteristics, clinical presentation, echocardiographic findings and outcomes were noted retrospectively. A binary logistic regression analysis using SPSS statistics software, version 25.0 was performed to establish myxoma features associated with atrioventricular valves obstruction. Results Mean age was 63±12 years old, 75% female. All CM were sporadic. The majority of the patients (52.4%, n=44) were asymptomatic, embolic events were present in 22.9% of the patients (n=19), with the most common site being central nervous system (19.0%, n=16) and constitutional symptoms in 16.7% (n=14), the most frequent fatigue (11.9%, n=10). Atrial fibrilation was present in 9.6% of the patients (n=8). The majority of the myxomas were located in the left atrium (88.1%), followed by right atrium (10.7%) and right ventricle (1.2%). Mean myxoma dimension was 36.8±17.4mm (longer axis), with heterogeneous aspect in 63.1%, presence of calcification in 11.9%, pediculated insertion in 40.5%, mobility in 70.2% and irregular borders in 52.4%. Moderate or severe ventricular inflow obstruction was present in 13.1% (n=11), 13.6% with mitral obstruction in the left atrial myxomas and 11.1% with tricuspid obstruction in the right atrial myxomas. CM longer axis 40–59mm and superior to 60mm were independently associated with obstruction (OR 5.1, 95% CI 1.4–18.4, p0.014 and OR 11.5, 95% CI 2.9–45.4, p0.001, respectively). No other morphologic features were associated with mitral or tricuspid obstruction. All but 3 patients had the myxoma surgically removed because of advanced age and comorbidities (96.4% surgical rate). There was no in-hospital or 1 year mortality in these patients. During the follow-up period, 3.6% patients had recurrence of the myxoma, with a men period of 5 years after the first surgery (one, two and twelve years), all of them re-submitted to surgical excision. Conclusion Despite being asymptomatic in the majority of the patients and often diagnosed incidentally, cardiac myxomas are frequent source of morbidity and mortality due to embolic events, obstructive and constitutional symptoms. Surgery is the treatment of choice for cardiac myxomas and in most cases it is curative, with low complications and low recurrence rate. Funding Acknowledgement Type of funding sources: None.

Fetal Atrial Septal Aneurysm: Follow-Up from Second to Third Trimester

Atrial septal aneurysm (ASA) is a rarely reported fetal finding. Its definition is variable, but the diagnosis is usually made when the foramen ovale flap extends at least halfway across the left atrium. It is considered a transient, self-limiting condition, but on occasion, it can be complicated by fetal arrhythmia or left ventricular (LV) inflow obstruction—if longstanding, this can lead to left heart hypoplasia. We present two cases of ASA diagnosed at the second trimester scan, one of which was subsequently complicated by LV inflow obstruction and prenatal suspicion of hypoplastic aortic arch. This report is a good illustration of how structure follows function: a small LV preload can lead to a decreased LV output, which in turn will end up in a hypoplastic LV and outflow tract—all this is reversible after birth, due to the physiological circulatory modifications that occur in the newborn.

Unusual cause of tricuspid valve obstruction after surgical aortic valve replacement.

Unruptured sinus of Valsalva aneurysm can present with manifestations due to local compression or protrusion into the chambers. Right ventricular inflow obstruction is a rare manifestation. This image highlights the tricuspid valve obstruction due to a Valsalva aneurysm in a patient after surgical aortic valve replacement.

Rare Case of an Adult With Double-Chambered Left Ventricle.

The rare case of an adult with a double-chambered left ventricle was revealed using multimodality imaging using echocardiography and cardiac magnetic resonance imaging in a 38-year-old asymptomatic male patient. The congenital malformation was dominated by a second, coarsely trabeculated muscular shelf dividing the left ventricle into 2 chambers without signs for left ventricular inflow or outflow tract obstruction. The partition wall did not show any signs for intramyocardial fibrosis in late gadolinium enhancement cardiovascular magnetic resonance imaging. Flow measurements excluded a relevant intracardial shunt across the additive perimembranous ventricular septal defect. There were no signs for global right and left ventricular dysfunction with left and right ventricular volumes and ejection fraction within normal limits. A conservative approach was recommended. In summary, we are able to present the case of an adult with a double-chambered left ventricle with a second muscular "septum" partially dividing the left ventricular cavity without causing a relevant impact on cardiac function or clinical signs for heart failure.

Giant Obstructive Myxoma with Hemolytic Anemia and Multiple Nevi in a 10-Year-Old Girl

Myxomas are low-prevalence heart tumors in the pediatric population. They are located in the left atrium in 75-86% of cases, and 30-50% are accompanied by general symptoms such as fever, weight loss, myalgias, arthralgias and elevated inflammatory markers caused by IL-6 release. They are rapidly growing heart tumors, and their most important complications are valvular and ventricular inflow tract obstruction, which may cause sudden death. Three to ten percent of cases are associated with Carney complex (CNC), type 1, caused by a mutation of the PRKAR1A gene on chromosome 17.

Left ventricular inflow obstruction due to a coronary arteriovenous fistula: a paediatric case report

BackgroundWe report a rare case of left ventricular inflow obstruction from a branch of the left circumflex coronary artery to the right atrium caused by a coronary arteriovenous fistula (CAVF) in a young Japanese male child.Case presentationThe patient was diagnosed with CAVF following a heart murmur shortly after birth. The left-to-right shunt caused right ventricular volume overload and pulmonary congestion. An emergency surgical intervention was performed for the CAVF on day 6 after birth. However, by 5 years of age, his left ventricular inflow obstruction worsened. We found an abnormal blood vessel originating from the proximal part of a branch of the left circumflex coronary artery, circling the outside of the mitral valve annulus along the medial side of the coronary sinus. As the child gets older, the blood inflow into the left ventricle might get restricted further, resulting in left-sided heart failure.ConclusionOur findings suggest that even after CAVF closure surgery, it is essential to monitor for complications caused by progressive dilatation of a persistent CAVF.

Giant Left Atrial Myxoma and Left Ventricular Inflow Obstruction.

Giant Left Atrial Myxoma and Left Ventricular Inflow Obstruction.

A Giant Left Atrial Myxoma Causing Left Ventricular Inflow Obstruction and Mitral Regurgitation

A Giant Left Atrial Myxoma Causing Left Ventricular Inflow Obstruction and Mitral Regurgitation

Right ventricular inflow obstruction related to late Candida albicans infection of implantable cardioverter-defibrillator leads.

Right ventricular inflow obstruction related to late Candida albicans infection of implantable cardioverter-defibrillator leads.

Left ventricular inflow obstruction and shock due to herniated peritoneal sac filled with ascites

Left ventricular inflow obstruction and shock due to herniated peritoneal sac filled with ascites

Metastatic melanoma of the heart: A systematic review.

e22017 Background: Melanomas are highly aggressive tumors that can metastasize to a wide array of organs, including the heart. Cardiac metastasis is rare and most often diagnosed post-mortem. Here, we perform a systematic review evaluating patient demographics, characteristics, management, and outcomes of cardiac melanoma metastases. Methods: A comprehensive literature review was performed in Cochrane Library, PubMed, and EmBase databases. Study quality was determined based on study design and clinical endpoints using the Oxford Center for Evidence Based Medicine (OCEBM) Levels of Evidence categorization. Demographic data, patient symptomatology, imaging findings, management strategies, and patient outcomes were collected and analyzed. Results: Thirty studies, all case reports or case series, met inclusion criteria. In total, 33 patients with cardiac metastasis from melanoma were identified and histologically confirmed. All 30 studies included in this review received an evidence level of 4 based on OCEBM Levels of Evidence categorization. The most common presenting symptom at the time of diagnosis of cardiac metastasis was shortness of breath (36.4%) and the most common physical exam finding was tachycardia (24.2%). The most common site of cardiac metastasis was the left ventricle (41.9%), followed by the right atrium (35.5%). Notable echocardiogram findings included right ventricular inflow obstruction (27.6%) and valvular dysfunction (24.1%). Valvular dysfunction, classified as prolapse, regurgitation, or stenosis, always involved the tricuspid valve. Treatment strategies typically involved surgical intervention (66.7%), chemotherapy (39.4%), or chemotherapy with surgical intervention (15.2%). Of the 22 cases that reported outcomes, the majority (59.1%) were disease-free without evidence of recurrence. Two patients (9.1%) experienced recurrence within one year of treatment and eight patients (36.4%) expired within 12 months. Conclusions: When symptomatic, cardiac melanoma metastases typically present with shortness of breath and tachycardia, with a predilection for the left ventricle and right atrium. Surgical intervention is currently the mainstay of treatment for successful cardiac melanoma metastasis management. [Table: see text]

A Rare Case of Epicardial Lead Strangulation of Left Ventricular Inflow

This is an unusual case of left atrioventricular groove strangulation by an abandoned epicardial pacing lead associated with mild left ventricular inflow obstruction, left atrial enlargement, and new atrial tachycardia that resolved with surgical lead removal. (Level of Difficulty: Intermediate.)

1636 Endomyocardial biopsy of right atrial mass with unfortunate consequence of right-to-left shunt

Abstract Funding Acknowledgements Nil A 43-year old lady complained of dyspnoea with elevated jugular venous pressure. Transthoracic echocardiography (TTE) showed massive pericardial effusion [Figure 1A] with a huge heterogeneous mass (5x9cm) attached to right atrial free wall [Figure 1B]. Therapeutic pericardiocentesis yielded blood-stained pericardial fluid with negative for tumour cells. Endomyocardial biopsy (EMB) was performed under transthoracic echocardiography (TTE) and fluoroscopy guidance. Right after procedure, there was a significant desaturation which could not be reversed by high flow oxygen supplement. The acute hypoxaemia was secondary to right-to-left shunt through a wide-open patent foramen ovale (PFO) as shown by transoesophageal echocardiography (TOE) [Figure 2A-D]. Transcatheter PFO closure was performed under TOE guidance with immediate correction of hypoxemia. [Figure 3]. Subsequent Computer Tomography demonstrated a large vascular mass at right atrium abutted onto the basal right ventricle and tricuspid valve [Figure 4] without evidence of tumor embolism. The histological diagnosis confirmed it was a cardiac sarcoma. Given the infiltrative nature of cardiac sarcoma with significant right ventricular (RV) inflow obstruction, a debulking surgery was done rather than a complete curative resection. She was referred to oncologist for adjuvant chemotherapy. We postulated that there is a shift in tumour location, i.e. more towards the tricuspid valve orifice which results in elevated right ventricular inflow obstruction which triggers the acute right-to-left shunting through PFO. Abstract 1636 Figure. EMB

A Man with a Clot on his Platelets: Adrenal Carcinoma and Dynamic Right Ventricular Inflow Obstruction

A Man with a Clot on his Platelets: Adrenal Carcinoma and Dynamic Right Ventricular Inflow Obstruction

Guz lewej komory serca u nastolatka

Asymptomatic 17-years-old boy with a suspicion of the heart tumor was admitted to the Cardiology Department for further investigation. Due to the murmur over the heart echocardiography was performed revealing a bright mass near the left ventricular apex. He was in a good physical condition, no symptoms like chest pain, shortness of breath, syncope, palpitations were reported. On physical examination diminished heart sounds and systolic murmur 2-3/6 in the Levin’s scale over the heart were found. Laboratory data including troponin T, CK, CK-MB, NSE, urea acid, marker were within normal limits. On ECG abnormalities of the repolarization were recorded (ST segment depression and T waves inversion in the II, III, aVF leads). Transthoracic echocardiogram revealed a 4.7 x 5.8 cm tumor within the postero-lateral wall of the left ventricle, without left ventricular inflow nor outflow obstruction. Based on cardiac magnetic resonance hemangioma supplied from the right coronary artery was suspected. Cardiac catheterization was performed, the left ventricular mass with supply from vascular network of the right coronary artery was confirmed. No abnormalities of the central nervous system in magnetic resonance were found. Full-body positron emission tomography showed cardiac mass without any evidence of extra-cardiac fluorodeoxyglucose-avid disease. Cardiac hemangioma was recognized. Pharmacological treatment with propranolol was introduced without any impact on the tumor size during 6 months follow-up.

Perioperative management of critical right ventricular inflow obstruction from right atrial rhabdomyoma

Rhabdomyoma is the most common cardiac tumor in infancy and commonly located in the ventricles causing outflow obstruction or arrhythmias. We report a rare pediatric (7 month old) case of a right atrial rhabdomyoma presenting with severe cyanosis and low cardiac output from significant tricuspid inflow obstruction with right to left shunt across a stretched patent foramen ovale. We present an emergency cardiac surgery for right atrial tumor resection, and the management of separating the patient with failing right ventricle from cardiopulmonary bypass using a Glenn shunt, since extracorporeal membrane oxygenation (ECMO) or nitric oxide was not available.

Primary epithelioid leiomyosarcoma of left atrium: Pathologist clinches it all

Primary cardiac neoplasms are rare with an estimated prevalence of 0.001%–0.2%. Cardiac tumors are mostly benign and myxomas are the commonest. Malignant tumors are rare in which metastasis and infiltration from the adjacent malignant tumor is more common than the primary ones. Amongst the primary malignant tumors sarcomas are the commonest. Primary epithelioid leiomyosarcoma of the heart is extremely rare. Here, we present a case of a 67-year-old female patient who was admitted with acute onset of pulmonary edema; on investigation, the patient was found to have left atrial mass. A provisional diagnosis of large left atrial myxoma with left ventricular inflow obstruction was made. Biopsy taken showed features of leiomyosarcoma which was confirmed by immunohistochemistry. Most of the patients with cardiac sarcoma have a high-grade tumor at the time of diagnosis with distant metastasis in the lungs (35.7%), lymph node (14.2%) followed by liver (7.14%) and they potent a poor prognosis. At present, only a few hundred primary cardiac sarcomas have been reported, most of which are based on autopsy series. Primary cardiac epithelioid leiomyosarcoma is a rare entity that can pose a diagnostic dilemma. However, atypical presentation of “atrial myxoma” being the common differential diagnosis. Histopathological evaluation is confirmatory to diagnose such a lesion. The pathologist should be aware of such an entity which can be attended by morphological evaluation including immunohistochemistry.