Search Results (14)

Tricuspid atresia (TA) is a cyanotic congenital heart defect defined by agenesis of the tricuspid valve and resultant right ventricular hypoplasia, representing 1.4–2.9% of congenital heart disease. Survival depends on interatrial and interventricular shunts that permit systemic and pulmonary blood flow, with staged surgical palliation culminating in the Fontan procedure. While surgical advances have improved long-term outcomes, Fontan circulation remains a delicate physiology characterized by preload dependence, elevated pulmonary vascular resistance, chronic venous hypertension, and a prothrombotic state. These features predispose patients to arrhythmias, lymphatic complications, hepatic congestion, and progressive circulatory failure. For anesthesiologists, perioperative management of TA and Fontan patients is uniquely complex. Anesthetic considerations include meticulous preload optimization, modulation of systemic and pulmonary vascular resistance, and ventilatory strategies that minimize adverse effects on venous return. Additional challenges include the high risk of air embolism, individualized anticoagulation needs, and hemodynamic sensitivity to patient positioning. Preoperative evaluation with echocardiography and electrocardiography provides critical insight into anatomy and physiology, while intraoperative planning must emphasize goal-directed fluid management, careful agent selection, and tailored ventilation. Postoperatively, vigilant monitoring, effective pain control, and prevention of complications are essential. This review synthesizes classification systems, pathophysiology, and the evolution of surgical palliation, while emphasizing anesthetic principles for the perioperative care of patients with TA and Fontan circulation. As survival improves and the population of Fontan patients expands, a nuanced understanding of this physiology is essential for optimizing outcomes across cardiac and non-cardiac surgical settings. Full article

An atrial septal defect (ASD) is the most common congenital heart defect (CHD) diagnosed in adulthood. It is characterized by significant anatomical heterogeneity and complications that evolve over time. While often asymptomatic in children, the signs of adverse effects of ASD increase with age, including a greater risk of heart failure, stroke, atrial fibrillation (AF), and reduced life expectancy. ASD is traditionally considered a right-heart lesion due to long-term complications such as arrhythmias, right-sided heart failure, thromboembolism, and, in a subset of patients, pulmonary arterial hypertension (PAH). The pathophysiology of atrial shunts also affects the left heart due to volume overload and adverse ventriculo-ventricular interaction. Early diagnosis of interatrial septal anomalies is essential to prevent hemodynamic consequences and/or thromboembolic events. Electrocardiographic (ECG) findings play a crucial role in this early diagnosis. This narrative review aims to update clinicians on the latest evidence regarding the pathophysiological link between ASD and cardiac rhythm disorders, the nuances of optimal diagnostics, treatment options (surgical, interventional, pharmacological), and the need for long-term follow-up for patients with ASD. The review will determine the risk of conduction disorders compared to a healthy population and to compare the prevalences of conduction disorders, mortality, and pacemaker use in patients with closed ASDs versus those with open ASDs. Full article

Background/Objectives: It is increasingly realized that the advances in diagnosis and treatment for those born with congenitally malformed hearts have now resulted in avoidance of morbidity being equally as important as avoiding postoperative mortality. Detailed personalized diagnoses will now be key to achieve such improvements. Methods: We have reviewed our own experience in diagnosing major phenotypic variations on selected congenital cardiac malformations, showing that the ability to personalize the findings is at hand, although not always to date universally employed. Results: We have chosen four categories to illustrate how the definitions now provided by the International Nomenclature Society, and incorporated in the 11th iteration of the International Classification of Disease, make it possible to provide personalized diagnoses. The lesions chosen for review are the arrangement of the atrial appendages, the lesions permitting interatrial shunting, the options in the setting of deficient ventricular septation, and the abnormal morphology of the aortic root. We show that not all centers, as yet, are taking advances of these opportunities at hand to tailor the chosen treatments. Conclusions: Detailed phenotypic definitions have now been provided for all the major congenital cardiac malformations. Use of these definitions should now provide personalized medicine for all those born with malformed hearts. As yet, the definitions are not used to their full effect. Full article

Background: The purpose of this study is the evaluation of imaging findings of acute-phase cardiac CT (cCT) in stroke patients with large vessel occlusion (LVO) to identify potential cardioembolic sources (CES) in patients without intracardiac thrombi and atrial fibrillation (AF). Material and Methods: This retrospective study included 315 patients with LVO who underwent cCT imaging in the acute stroke setting. The images were analysed for 15 imaging findings following the established minor and major cardioembolic risk factors. The final stroke aetiology was determined using the TOAST classification through interdisciplinary consensus following a thorough clinical evaluation. Multivariate regression analysis was performed to identify imaging findings associated with CES. Results: A cardioembolic aetiology was identified on cardiac CT in 211 cases (70%). After adjustment for AF and intracardiac thrombi, the multivariate regression analysis revealed significant associations with left ventricular dilation (adjusted odds-ratio (AOR) 32.4; 95% CI 3.0–349; p = 0.004), visible interatrial right-to-left shunt (AOR 30.8; 95% CI 2.7–341.3; p = 0.006), valve implants (AOR 24.5; 95% CI 2.2–270.9; p = 0.009), aortic arch atheroma grade > II (AOR 6.9; 95% CI 1.5–32.8; p = 0.015) and post-ischaemic myocardial scars (AOR 6.3, 95% CI 1.2–34.1; p = 0.032) as independent risk factors for a cardioembolic aetiology. The combined model achieved an area under the ROC curve of 0.83. Conclusions: In patients with LVO without AF and intracardiac thrombi as a cause, the presence of left ventricular dilatation, interatrial right-to-left shunts, valve implants, post-ischaemic myocardial scarring and advanced aortic arch atheroma (grade > 2) in particular is significantly associated with a cardioembolic cause of stroke and should be add-on evaluated in acute-phase cCT. Further investigations are warranted to confirm these associations. Full article

Sinus venosus atrial septal defects (SVASDs), concurrent with partial anomalous pulmonary venous connections (PAPVCs), are a rare congenital heart disease in dogs. Surgical correction is essential when clinical signs or significant hemodynamic changes are present. We aimed to report on the successful surgical correction of an SVASD with PAPVCs, using a computed tomography (CT)-based customized 3D cardiac model. A 10-month-old male poodle was referred for corrective surgery for an ASD. Echocardiography confirmed a hemodynamically significant left-to-right shunting flow through an interatrial septal defect and severe right-sided heart volume overload. For a comprehensive diagnosis, a CT scan was performed, which confirmed an SVASD with PAPVCs. A customized 3D cardiac model was used for preoperative decision-making and surgical rehearsal. The defect was repaired using an autologous pericardial patch under a cardiopulmonary bypass (CPB). Temporary pacing was applied for sinus bradycardia and third-degree atrioventricular block. The patient recovered from the anesthesia without further complications. The pacemaker was removed during hospitalization and the patient was discharged without complications 2 weeks post-surgery. At the three-month follow-up, there was no shunting flow in the interatrial septum and the right-sided volume overload had been resolved. The cardiac medications were discontinued, and there were no complications. This report indicates the validity of surgical correction under CPB for an SVASD with PAPVCs, and the advantages of utilizing a CT-based 3D cardiac model for preoperative planning to increase the surgical success rate. Full article

Traditional diagnosis of patent foramen ovale (PFO) in the heart has involved the use of transcranial Doppler (TCD). However, TCD is essentially a blind test that cannot directly visualize the location of blood vessels. Since TCD relies on qualitative assessments by examiners, there is room for errors, such as misalignment of the ultrasound’s angle of incidence with the actual blood vessels. This limitation affects the reproducibility and consistency of the examination. In this study, we presented an alternative approach for assessing right-to-left shunt (RLS) associated with PFO using contrast transcranial color-coded Doppler (C-TCCD) with bubble contrast. The patient under consideration had been diagnosed with an ischemic stroke through imaging, but the subsequent cardiac work-up failed to determine the cause. Employing C-TCCD for RLS screening revealed a confirmed RLS of Spencer’s three grades. Subsequently, transesophageal echocardiography (TEE) was conducted to evaluate PFO risk factors, confirming an 8 mm PFO size, a 21 mm tunnel length, a hypermobile interatrial septum, and persistent RLS. The calculated high-risk PFO score was 4 points, categorizing it as a very high risk PFO. This case underscores the importance of C-TCCD screening in detecting RLS associated with PFO, especially in cryptogenic stroke patients, when identifying the underlying cause of ischemic stroke becomes challenging. Full article

Patent foramen ovale (PFO) is a common cardiac abnormality with a prevalence of 25% in the general population. PFO has been associated with the paradoxical embolism causing cryptogenic stroke and systemic embolization. Results from clinical trials, meta-analyses, and position papers support percutaneous PFO device closure (PPFOC), especially if interatrial septal aneurysms coexist and in the presence of large shunts in young patients. Remarkably, accurately evaluating patients to refer to the closure strategy is extremely important. However, the selection of patients for PFO closure is still not so clear. The aim of this review is to update and clarify which patients should be considered for closure treatment. Full article

Infective endocarditis (IE) is common in patients with corrected congenital heart disease (CHD) with a residual lesion, but is rarely found on surgical patches used to close atrial septal defects (ASDs). This is also reflected in the current guidelines that do not recommend antibiotic therapy for patients with a repaired ASD with no residual shunt six months after closure (percutaneous or surgical). However, the situation could be different in the case of mitral valve endocarditis, which causes leaflet disruption with severe mitral insufficiency and could seed the surgical patch. We present herein a 40-year-old male patient with a past medical history of a complete surgically corrected atrioventricular canal defect performed in childhood who presented with fever, dyspnea and severe abdominal pain. Transthoracic and transesophageal echocardiography (TTE and TEE) revealed vegetation at the level of the mitral valve and the interatrial septum. The CT scan confirmed ASD patch endocarditis and multiple septic emboli, guiding the therapeutic management. An accurate evaluation of cardiac structures should be mandatory when a systemic infection is detected in CHD patients, even if the defects were surgically corrected, because the detection and eradication of such infectious foci as well as a surgical reintervention are particularly difficult to achieve in this subpopulation. Full article

Diamond–Blackfan anemia is a rare (6–7 million live births), inherited condition manifesting as severe anemia due to the impaired bone marrow production of red blood cells. We present the unusual case of a six month old infant with a de novo mutation of the RPS19 gene causing Diamond–Blackfan anemia who additionally suffers from severe sinus bradycardia. The infant was diagnosed with this condition at the age of four months; at the age of 6 months, she presents with severe anemia causing hypoxia which, in turn, caused severe dyspnea and polypnea, which had mixed causes (hypoxic and infectious) as the child was febrile. After correction of the overlapping diarrhea, metabolic acidosis, and severe anemia (hemoglobin < 3 g/dL), she developed severe persistent sinus bradycardia immediately after mild sedation (before central venous catheter insertion), not attributable to any of the more frequent causes, with a heart rate as low as 49 beats/min on 24 h Holter monitoring, less than the first percentile for age, but with a regular QT interval and no arrhythmia. The echocardiogram was unremarkable, showing a small interatrial communication (patent foramen ovale with left-to-right shunting), mild left ventricular hypertrophy, normal systolic and diastolic function, and mild tricuspid regurgitation. After red cell transfusion and appropriate antibiotic and supportive treatment, the child’s general condition improved dramatically but the sinus bradycardia persisted. We consider this a case of well-tolerated sinus bradycardia and foresee a good cardiologic prognosis, while the hematologic prognosis remains determined by future corticoid response, treatment-related complications and risk of leukemia. Full article

Concomitant systemic essential hypertension (HTN) in adults with a secundum atrial septal defect (ASD) can unfavorably affect the hemodynamics and transcatheter ASD closure (ASDC) effects. This study aims to assess the effectiveness and safety of ASDC in adults with HTN in real-world clinical practice. Right ventricular (RV) reverse remodeling (RVR) and the lack of a left-to-right interatrial residual shunt (NoRS) in echocardiography 24 h and 6 months (6 M) post-ASDC, and ASDC-related complications within 6 M were evaluated in 184 adults: 79 with HTN (HTN+) and 105 without HTN (HTN−). Compared to HTN−, HTN+ patients were older and had a greater RV size and the prevalence of atrial arrhythmias, chronic heart failure, nonobstructive coronary artery disease, diabetes, hyperlipidemia, and left ventricular diastolic dysfunction. ASDC was successful and resulted in RVR, NoRS, and a lack of ASDC-related complications in the majority of HTN+ patients both at 24 h and 6 M. HTN+ and HTN− did not differ in ASD size, a successful implantation rate (98.7% vs. 99%), RVR 24 h (46.8% vs. 46.7%) and 6 M (59.4% vs. 67.9%) post-ASDC, NoRS 24 h (79% vs. 81.5%) and 6 M (76.6% vs. 86.9%) post-ASDC, and the composite of RVR and NoRS at 6 M (43.8% vs. 57.1%). Most ASDC-related complications in HTN+ occurred within 24 h and were minor; however, major complications such as device embolization within 24 h and mitral regurgitation within 6 M were observed. No differences between HTN+ and HTN− were observed in the total (12.7% vs. 9.5%) and major (5.1% vs. 4.8%) complications. Transcatheter ASDC is effective and safe in adults with secundum ASD and concomitant HTN in real-world clinical practice; however, proper preprocedural management and regular long-term follow-up post-ASDC are required. Full article

Invasive interventions have been conducted in preterm infants with significant patent ductus arteriosus (PDA) when medical treatment has failed, and methods of invasive intervention have been reported. Surgical ligation via lateral thoracotomy has been a well-established procedure for decades. Recently, transcatheter occlusion has been safely and feasibly applied to the premature population. However, little research has been conducted on the benefits of transcatheter occlusion in very-low-birth-weight (VLBW) infants compared to surgical ligation. This study compared transcatheter and surgical techniques in VLBW infants in terms of short-term respiratory outcomes. The medical records of 401 VLBW infants admitted to a tertiary hospital between September 2014 and January 2019 were retrospectively reviewed. Patients who were diagnosed with a congenital anomaly, a chromosomal anomaly, or congenital heart disease, except for an inter-atrial shunt, were excluded. The perinatal conditions, neonatal morbidities, periprocedural vital signs, and respiratory support trajectories were compared between the transcatheter-treated and surgically ligated group. A total of 31 eligible VLBW infants received invasive intervention: 14 were treated with transcatheter occlusion (Group A), and 17 infants were treated with surgical ligation (Group B). Respiratory outcomes were not statistically significant between the two groups, despite Group A showing a trend toward early improvement in post-intervention respiratory trajectory. In this small case study, a different trend in post-intervention respiratory trajectories was observed. Future research with larger case numbers should be conducted to address our preliminary observations in more detail. Full article

The aim of this study was to analyze results of stenting atrioseptostomy in patients with pulmonary arterial hypertension and a different level of risk for one-year mortality that is not well described. Patients that underwent atrioseptostomy with stenting were retrospectively divided in two groups: “intermediate” (n = 55) or “high” risk (n = 13), according to the 2015 ESC/ESR guideline. Results of atrioseptostomy were assessed during hospital period and at follow-up. Patients from “intermediate” risk group demonstrated lower mortality rate (10/55, vs. 6/13) during the course of the study period, as well as higher freedom from lung transplantation or Potts shunt. At discharge, patients of both groups presented improvement in functional class and mobility. Patients from “intermediate” risk group showed longer 6-min walking distance, and lower levels of brain natriuretic peptide. At the latest follow-up, stable position and full patency of stents with right-to-left or bidirectional shunt at atrial level and absence of syncope was confirmed in patients of both groups. Patients from the “intermediate” risk group demonstrated higher functional class, better performance of walking test, and lower levels of brain natriuretic peptide. Stenting atrioseptostomy reliably secured interatrial communication and improved clinical condition in patients with idiopathic pulmonary arterial hypertension. Mid-term results were better in “intermediate” risk group. Full article

A 56-year old male with ischemic heart disease and an unremarkable preoperative echocardiogram underwent surgical coronary revascularization. An intraoperative post pump trans-esophageal echocardiogram (TOE) performed while the patient was being ventilated at a positive end expiratory pressure (PEEP) of 8 cm H₂O demonstrated a right to left interatrial shunt across a patent foramen ovale (PFO). Whereas oxygen saturation was normal, a reduction of the PEEP to 3 cm H₂O led to the complete resolution of the shunt with no change in arterial blood gases. Attempts to increase the PEEP level above 3 mmHg resulted in recurrence of the interatrial shunt. The remaining of the TEE was unremarkable. Mechanical ventilation, particularly with PEEP, causes an increase in intrathoracic pressure. The resulting rise in right atrial pressure, mostly during inspiration, may unveil and pop open an unrecognized PFO, thus provoking a right to left shunt across a seemingly intact interatrial septum. This phenomenon increases the risk of paradoxical embolism and can lead to hypoxemia. The immediate management would be to adjust the ventilatory settings to a lower PEEP level. A routine search for a PFO should be performed in ventilated patients who undergo a TEE. Full article

Platypnea-orthodeoxia syndrome (POS) is a rare condition characterised by episodes of dyspnoea and desaturation when changing from a recumbent to a sitting or standing position, due to a right-to-left shunt through the atrial septum (cardiac POS) or a pulmonary vascular shunt. A c onsistent finding among patients with cardiac POS is an interatrial communication (patent foramen ovale [PFO] or atrial septal defect [ASD]), but these often-congenital anatomies, alone, do not account for development of massive right-to-left shunt, which frequently occurs later in life leading to severe hypoxaemia. We present the case of an 84-year-old woman known for evolutive right breast carcinoma with successive loco-regional progressions, which over several years, despite local and systemic therapy, eventually invaded the right pectoral muscle, with skin infiltration of the right chest wall and right diaphragmatic paralysis. She subsequently developed rapidly progressive dyspnoea: a cardiac workup including contrast examinations (trans-cranial duplex and trans-thoracic echocardiography) showed a massive intra-cardiac right-to-left shunt, while transoesophageal echocardiography confirmed the presence of a large PFO. Based on these findings, the patient was diagnosed with POS and PFO closure was performed with immediate normalisation of oxygen saturation, and at 6–month follow- up no further episodes of desaturation were reported. The aim of this case discussion was to underline the mechanisms leading to significant right-to-left shunt among patients with PFO, especially when in an upright position, and to elucidate the therapeutic options. Full article