Intracardiac Shunts Research Articles

Intracardiac migration of distal catheter—a rare complication of VP shunt insertion: case report and literature review

Intracardiac migration is a rare complication of ventriculoperitoneal shunt insertion. Only 15 cases have been reported, 7 of which were paediatric cases, treated with techniques including interventional radiography, open thoracotomies and direct extraction through the initial shunt incision. The authors report the youngest case of intracardiac shunt migration complicated by significant coiling and knotting within the cardiac chambers and pulmonary vasculature. Migration likely began when the SVC was pierced during initial shunt placement and progressed due to negative intrathoracic pressure. Extrusion was achieved combining thoracoscopic endoscopy, interventional fluoroscopy screening and a posterolateral neck incision with uncoiling of the shunt via a Seldinger guide wire. This offered a minimally invasive solution with rapid post-operative recovery.

A pediatric case of portosystemic shunt presenting hypoxemia with brain abscess in an emergency department

Portosystemic shunt is a rare congenital vascular anomaly. The shunt is hard to clinically suspect because its symptoms develop insidiously and affect various systems at different time points. We report a case of an 11-year-old girl who presented with brain abscess and hypoxemia without respiratory distress. No abnormality related to the hypoxemia, such as intracardiac shunt, was found on the initial chest radiograph and echocardiogram. However, computed tomography showed a portosystemic shunt. An intrapulmonary shunt was demonstrated by bubble contrast echocardiography, indicating a grade 2 hepatopulmonary syndrome, a complication of portosystemic shunt. This case describes the diagnostic process of an unusual case presenting neurological abnormalities and hypoxemia without respiratory distress. Portosystemic shunt with hepatopulmonary syndrome should be considered in the differential diagnosis of unexplained hypoxemia.

Managing Congenital Heart Defects in Elderly: The Platypnea-Orthodeoxia Syndrome in Underestimated Patent Foramen Ovale.

The platypnea-orthodeoxia syndrome (POS) is a rare and often suboptimally managed condition with a complex diagnostic workup, conversely displaying an easy treatment and a good recovery of symptoms, especially if consequent to an intracardiac shunt. However, its identification is challenging, due to the several clinical manifestations, the multiple etiologies, representing often the delayed presentation of a congenital heart disease. We present a case report and review of available literature on patients with the POS secondary to a patent foramen ovale successfully treated with its closure.

Management of systemic to pulmonary shunts and elevated pulmonary vascular resistance.

Repair of systemic to pulmonary shunts is timed to prevent the development of irreversible pulmonary vascular disease, including in patients with other factors contributing to pulmonary hypertension. This study assessed outcomes of an individualised strategy for managing patients with mild-moderately elevated pulmonary vascular resistance (PVR) deemed borderline eligible for repair. A retrospective chart review was conducted of patients with systemic to pulmonary shunts and baseline indexed PVR (PVRi) ≥3 WU·m2 treated at a single centre from 1 January 2005 to 30 September 2019. Data included demographics, World Health Organization functional class (WHO FC), medications and haemodynamic data at baseline and serial follow-up. 30 patients (18 females) met criteria for inclusion. Median age at diagnosis of pulmonary arterial hypertension was 1.3 years (range 0.03-54 years) and at surgery was 4.1 years (range 0.73-56 years). Median follow-up time was 5.8 years (range 0.2-14.6 years) after repair. Most patients received at least one targeted pulmonary arterial therapy prior to repair and the majority (80%) underwent fenestrated shunt closure. There was a significant decrease in mean pulmonary arterial pressure (mPAP) (p<0.01), PVRi (p=0.0001) and PVR/systemic vascular resistance (p<0.01) between baseline and preoperative catheterisation and a decrease in PVRi (p<0.005), mPAP (p=0.0001) and pulmonary to systemic flow ratio (p<0.03) from baseline to most recent catheterisation. WHO FC improved from FC II-III at baseline to FC I post repair in most patients (p<0.003). In carefully selected patients with systemic to pulmonary shunts and elevated PVR considered borderline for operability, the use of preoperative targeted therapy in conjunction with fenestrated or partial closure of intracardiac shunts is associated with improvement in WHO FC and clinical outcomes.

Echocardiographic parameters recommended for assessing the severity of tricuspid regurgitation: concordance and discordance.

Current guidelines recommend integrating several echocardiographic indices to evaluate the severity of tricuspid regurgitation (TR). Discordance of indices, including qualitative and quantitative methods, commonly exists in practice. The discordance among these parameters has not yet been fully elucidated. A total of 127 patients with recognizable TR jets without pulmonary regurgitation or intracardiac shunt were prospectively enrolled. We evaluated 8 parameters by 2-dimensional (2D) echocardiography: proximal iso-velocity surface area (PISA)-derived regurgitant volume (RVol), PISA-derived effective regurgitant orifice area (EROA), PISA radius, vena contracta width (VCW), color Doppler jet area, tricuspid valve annular diameter, inferior vena cava (IVC) diameter, and peak E wave. According to current guidelines, each echocardiographic parameter was determined to represent either severe or non-severe TR. A concordance score was calculated as the number of concordant parameters divided by 8, with a higher score reflecting better concordance. Data were further categorized into 3 subgroups: complete concordance (0 discordant parameters), high concordance (1-2 discordant parameters), and low concordance (3-4 discordant parameters). The mean concordance score was 81%±17% for the entire cohort. There were 48 (38%) patients with complete concordance, including 6 patients with severe TR. In contrast, the low concordance group (n=43, 34%) mostly comprised severe TR patients (36 patients). When considering only EROA, RVol, and VCW, concordance improved, with 98 patients (77%) in complete agreement. Concordance seems limited when using echocardiographic parameters to assess TR severity. Applying only EROA, RVol, and VCW results in better concordance, as recommended by the current guidelines.

Anomaly of Systemic Venous Drainage With Left-to-Right Intracardiac Shunts

Anomaly of Systemic Venous Drainage With Left-to-Right Intracardiac Shunts

Intracardiac Shunt Assessment Using Ct Coronary Angiography

Intracardiac Shunt Assessment Using Ct Coronary Angiography

Thromboembolic diseases from a cardiological point of view

Thromboembolic disease is associated with a high mortality. It can be divided into two groups: embolism from a venous and embolism from an arterial side. This article gives an overview on thromboembolic disease (with a focus on pulmonary embolism and ischemic stroke) from a cardiologist's perspective.The therapeutic options for acute pulmonary embolism range from anticoagulation to fibrinolysis to interventional recanalization and surgery. The deciding factor for choice of therapy is the risk of early death. Besides clinical parameters, laboratory markers like cardiac troponin and right ventricular function on echocardiography or CTPA (computed tomography pulmonary angiography) are used to determine the early mortality risk. In hemodynamically instable patients, immediate thrombolysis is required, whereas patients with intermediate and low risk can be treated with anticoagulation. Interventional recanalization is currently being studied in patients at risk for development of CTEPH (chronic thromboembolic pulmonary hypertension) or an intermediate risk of early mortality.In ischemic stroke, early interdisciplinary workup involving a cardiologist is paramount. Post stroke screening should include monitoring for arrythmias (especially atrial fibrillation) and transthoracic echocardiography as well as sonography of extra- and intracranial arteries. If no embolic source can be detected (embolic stroke of undetermined source), transesophageal echo can be helpful to detect intracardiac shunts like patent foramen ovale (PFO) or intracardiac tumors. Post stroke care includes secondary prevention measures like risk factor modification and lipid lowering therapy as well as anticoagulation. In high risk for paradoxical embolization, interventional PFO closure can be performed. Interventional closure of the left atrial appendage (LAA) can be discussed in patients with both high thromboembolic and bleeding risk.

New application of saline contrast-enhanced electrical impedance tomography method for right ventriculography besides lung perfusion: detection of right-to-left intracardiac shunt.

Saline contrast-enhanced electrical impedance tomography (EIT) has been used to identify the respiratory failure etiologies through assessment of regional lung perfusion at the bedside. In this study, we introduce a novel approach to detect right-to-left intracardiac shunt based on the center of heart (CoH) parameter determined from the early phase of impedance-time curve after saline bolus injection. The timepoints when the saline bolus enter the heart (T0) and the lung regions (T1) are identified at first. A moving time window from T0 to T1 is then generated with steps of 0.5 s and the slope of the impedance-time curve in each pixel within the window calculated. CoH is calculated as the geometric center of pixel slope values in the right-to-left image direction. To illustrate how this method works in practice, we calculated the CoH values at T0 to T1 in 10 control hypoxic patients with no right-to-left shunt. In addition, we examined two critically ill patients with right-to-left intracardiac shunt. One was postcardiac surgery patient who had a residual atrial septal defect by color doppler of transesophageal echocardiograph. The other patient had a congenital heart disease of ventricular septal defect by color doppler of trans-thoracic echocardiography. A large difference in CoH between T0 to T1 was observed in the two patients with intracardiac shunt than in the control patients (11.06 ± 3.17% vs. 1.99 ± 1.43%, P = 0.030). Saline bolus EIT for lung perfusion might be used as ventriculography to identify the right-to-left intracardiac shunt at the bedside.

Positive Bubble Study But No Evidence of Interatrial Defect in a Patient with Recurrent Cryptogenic Stroke.

Pulmonary arteriovenous malformations (PAVMs) can be asymptomatic or result in a range of complications such as brain abscesses or cryptogenic emboli, which can contribute to morbidity and mortality if not diagnosed and treated in a timely manner. To date, there have been several reports of delayed diagnosis of PAVMs, which have been largely attributed to the misconception that PAVMs are too rare to be of clinical significance. Furthermore, because intracardiac shunting secondary to a patent foramen ovale (PFO) or atrial septal defect (ASD) also results in a positive saline contrast study with echocardiography, PAVM can be easily misdiagnosed as an intracardiac right-toleft shunt. However, there are unique echocardiographic features that differentiate between intracardiac shunting due to a PFO or ASD and extracardiac shunting such as in PAVM. This case details the course of a patient with recurrent cryptogenic strokes that was initially misattributed to a PFO and was only correctly diagnosed with multiple PAVMs after two failed attempts at PFO closure. This case serves as a reminder of an alternative etiology of right-to-left shunt and its presentation on imaging, which echocardiographers must be familiar with.

Patent Foramen Ovale Closure in a Nonagenarian With Platypnoea-Orthodeoxia Syndrome

Patent foramen ovale (PFO) is a common congenital cardiac abnormality that affects one-quarter of the general population. Hypoxaemia, because of large right-to-left shunt, is a rare complication of PFO and occurs under exceptional physiological conditions. Case: This 90-year-old lady presented with progressive dyspnoea over several months. At presentation to hospital, she was hypoxaemic with pO2 of 53 mmHg and oxygen saturation of 89% despite 15L of oxygen via a mask. While lying supine for a computed tomography pulmonary angiogram (CTPA), it was noted that her oxygen saturation normalised. The CTPA excluded pulmonary embolus and intrapulmonary vascular anomaly. Intermittent intracardiac shunt was suspected and confirmed on formal shunt testing with a shunt fraction calculated at 32%. Transthoracic echocardiogram and cardiac CT failed to demonstrate an intracardiac shunt. Transoesophageal echocardiogram confirmed a PFO with dynamic right-to-left shunting on colour Doppler and with agitated saline. She underwent percutaneous closure with a 25-mm Amplatzer PFO Occluder, with a good procedural result and resolution of her symptoms. At the 2-month follow-up she remained well with no breathlessness and no device-related complications. This case illustrates an unusual cause of platypnoea-orthodeoxia syndrome due to PFO in a nonagenarian. It was hypothesised that a prominent Eustachian valve, and anatomical factors including enlarged tortuous aorta and pectus excavatum, had gradually progressed over time, distorting the cardiac anatomy, and contributing to significant shunting. Percutaneous PFO closure resulted in immediate symptomatic benefit.

C47 ADAPTATION OF THE PULMONARY CIRCULATION AND OF THE RIGHT HEART IN INTRACARDIAC INVETERATE SHUNTS: UNDER PRESSURE OR LET IT FLOW?

Abstract Congenital heart disease can be associated with pulmonary hypertension (PH) development and heart right remodelling. Nevertheless, the adaptation of the pulmonary circulation and of the right heart can be extremely heterogeneous, so that an accurate diagnostic work up may be advisable when an intracardiac shunt is suspected. Case Presentation A 53 years old woman, with no relevant clinical history and asymptomatic, was incidentally found to have clinical, ECG and echocardiographic signs of PH, and was referred to our Center. Diagnostic and Therapeutic Work Up Echocardiography showed: normal left ventricle (LV 3D volumes 88/35 ml), mild left atrial dilatation (39 ml/m2); severe right chambers dilatation (RV 3D 347/124 ml, EF 3D 62%, AD 116 ml/m2), moderate tricuspid regurgitation with a regurgitant jet velocity of 2.9 m/s. Right heart catheterization showed normal pulmonary pressures at rest (mPAP 20, PAWP 5 mmHg), extremely high pulmonary blood flow (Qp 29 L/min, calculated with the direct Fick method), with normal pulmonary vascular resistance (PVR 0.5 WU). During the oxymetric run, a step up in hemoglobin saturation between superior vena cava and right atrium (from 74 to 97%) was found, with a Qp/Qs ratio of 5.5. During exercise, Qp reserve was normal, suggesting a normal contractile reserve function of RV despite the extreme dilatation. Additionally, the patient did not develop exercise PH and exercise capacity was normal, based on a measured oxygen consumption (VO2) at peak of 24 ml/kg/min (104% of predicted). Angio–CT of the chest was performed, confirming the presence of a sinus venosus atrial septal defect. The patient was then referred for surgical correction. Conclusion Echocardiography signs are useful to direct correctly diagnostic work up. A severe RV dilatation with normal systolic function in an asymptomatic middle age woman should raise the possibility of an intracardiac shunt. Despite a large intracardiac shunt with a high Qp/Qs ratio for decades, PVR was normal, confirming that individual predisposition may play a role in the development of PH in pre–tricuspid shunts. Exercise test showed an optimal RV reserve, suggesting a low cardiac surgery risk.

C13 AT THE ROOT OF HYPOXIA: NEVER UNDERESTIMATE THE CLINICAL

Abstract A 74–year–old woman arrived to the ER for syncope, reporting episodes of atypical chest pain for a few months. The initial ECG showed negative T waves in the lateral leads; an echocardiogram documented lower–limit systolic function due to akinesia of the antero–lateral segments and the presence of an ascending aorta aneurysm (AAA). The woman, with multiple cardiovascular risk factors, had previous transmural myocardial ischemia documented by MRI and never investigated with coronary angiography (ICA). Admitted to ICCU for suspected ischemic heart disease, she experienced a sudden clinical deterioration with onset of acute respiratory failure, initially stabilized with noninvasive ventilation. A chest angioCT excluded acute events (pulmonary embolism, aortic dissection) and confirmed the presence of AAA with bovine arch. Given the persistent need for oxygen therapy, closer clinical observation allowed objectification of platypnea–orthodeoxia syndrome (POS) that presented with desaturation, dyspnea, and mental confusion in the orthostatic position that improved with supine position. A bubble test in the two positions documented worsening intracardiac shunt in the sitting position, and a transesophageal echocardiogram showed the presence of a tunnel–like patent foramen ovale (PFO). The patient then underwent ICA that found a stenosis–free vascular tree, but a marked fusiform common trunk aneurysm (CTA) was appreciated. After exclusion of altered cardiopulmonary hemodynamic balance, percutaneous closure of the PFO was performed, which allowed progressive improvement of respiratory exchanges and subsequent complete weaning from oxygen. Typically, PFO occurs in the case of a pulmonary ventilo–perfusory mismatch or right–left shunt (rlS), associating mainly with atrial congenital defects and, to a lesser extent, pulmonary arteriovenous malformations. Sometimes, for POS to occur, the presence of a functional component facilitating rlS is necessary. In our case, it is conceivable that such a contribution was both ab–extrinsic compression of the right atrium by the AAA and compression of the pulmonary trunk at the level of the pulmonary valve by the CTA. This diagnostic hypothesis is compatible with the late presentation.

Catch bonds in sickle cell disease: Shear-enhanced adhesion of red blood cells to laminin

Could the phenomenon of catch bonding—force-strengthened cellular adhesion—play a role in sickle cell disease, where abnormal red blood cell (RBC) adhesion obstructs blood flow? Here, we investigate the dynamics of sickle RBCs adhering to a surface functionalized with the protein laminin (a component of the extracellular matrix around blood vessels) under physiologically relevant microscale flow. First, using total internal reflectance microscopy we characterize the spatial fluctuations of the RBC membrane above the laminin surface before detachment. The complex dynamics we observe suggest the possibility of catch bonding, where the mean detachment time of the cell from the surface initially increases to a maximum and then decreases as a function of shear force. We next conduct a series of shear-induced detachment experiments on blood samples from 25 sickle cell disease patients, quantifying the number and duration of adhered cells under both sudden force jumps and linear force ramps. The experiments reveal that a subset of patients does indeed exhibit catch bonding. By fitting the data to a theoretical model of the bond dynamics, we can extract the mean bond lifetime versus force for each patient. The results show a striking heterogeneity among patients, both in terms of the qualitative behavior (whether or not there is catch bonding) and in the magnitudes of the lifetimes. Patients with large bond lifetimes at physiological forces are more likely to have certain adverse clinical features, like a diagnosis of pulmonary arterial hypertension and intracardiac shunts. By introducing an in vitro platform for fully characterizing RBC-laminin adhesion dynamics, our approach could contribute to the development of patient-specific antiadhesive therapies for sickle cell disease. The experimental setup is also easily generalizable to studying adhesion dynamics in other cell types, for example, leukocytes or cancer cells, and can incorporate disease-relevant environmental conditions like oxygen deprivation.

To study about the relation between oxygen delivery and hemodynamics during cardiopulmonary bypass

The study put forth the relation between oxygen delivery and hemodynamics during cardiopulmonary bypass. The study was performed on 26 patients of either sex have age &gt; 10 years and who underwent elective open-heart surgery from June 2017 – May 2018 in Sri Ramachandra Medical College &amp; Research Institute. The patients with valvular heart diseases associated with other cardiac lesions like Intra cardiac shunts (ASD, VSD etc.) and Coronary artery disease (CABG) are excluded in the study. It examined the relationships among VO2, hemodynamics &amp; DO2 during clinical perfusion, namely throughout moderately hypothermic CPB using a non-pulsatile roller pump while managing the alpha-stat acid-base with adults. The results revealed a linear relationship between VO2 and DO2, demonstrating that higher levels of delivery can perfuse and collect additional vascular beds throughout the CPB. Under bodily tissue perfusion, higher deliveries are advised to be avoided. The oxygen demand decreases by 7% for every 1OC decline in patient temperature.

Unrecognized Patent Foramen Ovale in Patient With Sinus Venosus-Type Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return.

Unrecognized Patent Foramen Ovale in Patient With Sinus Venosus-Type Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return.

SPECIAL CONSIDERATIONS IN CRITICAL CARE OF THE CONGENITAL HEART DISEASE PATIENT.

Outcomes of congenital heart disease have improved markedly over the last 20 years, with survival to adulthood now close to 90%. The mean age of admission to an intensive care unit (ICU) is 40 years. The incidence of hospital and critical care admissions have increased significantly as a consequence of this improved survival. Intensivists are now confronted with the management of complex adult congenital heart disease (ACHD) lesions not only from a cardiac perspective but also extra-cardiac organ consequences of years of abnormal circulation following surgical or palliative correction. Kidney and liver dysfunction, respiratory and hematological abnormalities are very common in this population. ACHD patients can present to the ICU for a vast number of reasons, these are classified in this review as medical non-cardiac, medical cardiac and surgical. Community/hospital acquired infections, cerebrovascular accidents, respiratory failure, alongside arrhythmias and heart failure are responsible for medical admissions. Surgical admissions include post-operative management after correction or palliation, but also medical optimization and work-up for advanced therapies. ICU management of this large heterogeneous group requires a thorough understanding of the pathophysiology in order to apply conventional adult critical care modalities; left ventricular or right ventricular dysfunction, pulmonary hypertension, intracardiac, extracardiac, palliative surgical shunts can be present and require additional consideration. This review focuses on the pathophysiology, long term sequelae, and different treatment modalities to supply a framework for the ICU physician caring for these patients. Successful outcome, especially in complex lesions is dependent on early involvement of specialized ACHD centers.

A multidisciplinary approach to severe bronchopulmonary dysplasia is associated with resolution of pulmonary hypertension.

To describe our multidisciplinary bronchopulmonary dysplasia (BPD) consult team's systematic approach to BPD associated pulmonary hypertension (PH), to report our center outcomes, and to evaluate clinical associations with outcomes. Retrospective cohort of 60 patients with BPD-PH who were referred to the Seattle Children's Hospital BPD team from 2018 to 2020. Patients with critical congenital heart disease were excluded. Demographics, comorbidities, treatments, closure of hemodynamically relevant intracardiac shunts, and clinical outcomes including time to BPD-PH resolution were reviewed. Median gestational age of the 60 patients was 25 weeks (IQR: 24-26). 20% were small for gestational age (SGA), 65% were male, and 25% received a tracheostomy. With aggressive cardiopulmonary management including respiratory support optimization, patent ductus arteriosus (PDA) and atrial septal defect (ASD) closure (40% PDA, 5% ASD, 3% both), and limited use of pulmonary vasodilators (8%), all infants demonstrated resolution of PH during the follow-up period, including three (5%) who later died from non-BPD-PH morbidities. Neither SGA status nor the timing of PH diagnosis (<36 vs. ≥36 weeks PMA) impacted the time to BPD-PH resolution in our cohort [median 72 days (IQR 30.5-166.5)]. Our multidisciplinary, systematic approach to BPD-PH management was associated with complete resolution of PH with lower mortality despite less sildenafil use than reported in comparable cohorts. Unique features of our approach included aggressive PDA and ASD device closure and rare initiation of sildenafil only after lack of BPD-PH improvement with respiratory support optimization and diagnostic confirmation by cardiac catheterization.

Can Isolated Discordant Atrioventricular Connections Survive Without Interatrial or Interventricular Shunt?

Clinical presentation of isolated discordant atrioventricular connections is akin to transposition of the great arteries. In the absence of a significant intracardiac shunt, profound cyanosis is expected at birth. We report one such 5-month-old infant who had only mild cyanosis. The left-sided tricuspid valve straddled the interventricular septum with a closed interventricular communication, a type of "Double Outlet Left Atrium with three atrioventricular valves," which provided the necessary "left to right" shunt while severe regurgitation through the straddling part and a patent ductus arteriosus provided the effective pulmonary blood flow.

Hemodynamic Effects of a High-Frequency Oscillatory Ventilation Open-Lung Strategy in Critically Ill Children With Acquired or Congenital Cardiac Disease.

To study the hemodynamic consequences of an open-lung high-frequency oscillatory ventilation (HFOV) strategy in patients with an underlying cardiac anomaly with or without intracardiac shunt or primary pulmonary hypertension with severe lung injury. Secondary analysis of prospectively collected data. Medical-surgical PICU. Children less than 18 years old with cardiac anomalies (± intracardiac shunt) or primary pulmonary hypertension. None. Data from 52 subjects were analyzed, of whom 39 of 52 with cardiac anomaly (23/39 with intracardiac shunt) and 13 of 52 with primary pulmonary hypertension. Fourteen patients were admitted postoperatively, and 26 patients were admitted with acute respiratory failure. Five subjects (9.6%) were canulated for ECMO (of whom four for worsening respiratory status). Ten patients (19.2%) died during PICU stay. Median conventional mechanical ventilation settings prior to HFOV were peak inspiratory pressure 30 cm H 2 O (27-33 cm H 2 O), positive end-expiratory pressure 8 cm H 2 O (6-10 cm H 2 O), and F io2 0.72 (0.56-0.94). After transitioning to HFOV, there was no negative effect on mean arterial blood pressure, central venous pressure, or arterial lactate. Heart rate decreased significantly over time ( p < 0.0001), without group differences. The percentage of subjects receiving a fluid bolus decreased over time ( p = 0.003), especially in those with primary pulmonary hypertension ( p = 0.0155) and without intracardiac shunt ( p = 0.0328). There were no significant differences in the cumulative number of daily boluses over time. Vasoactive Infusion Score did not increase over time. Pa co2 decreased ( p < 0.0002) and arterial pH significantly improved ( p < 0.0001) over time in the whole cohort. Neuromuscular blocking agents were used in all subjects switched to HFOV. Daily cumulative sedative doses were unchanged, and no clinically apparent barotrauma was found. No negative hemodynamic consequences occurred with an individualized, physiology-based open-lung HFOV approach in patients with cardiac anomalies or primary pulmonary hypertension suffering from severe lung injury.