Restrictive Atrial Communication Research Articles

Surgical outcome of the borderline hypoplastic left ventricle: impact of the left ventricle rehabilitation strategy.

This study aims to assess the surgical outcome of borderline hypoplastic left ventricle before and after the induction of the left ventricle rehabilitation strategy. A retrospective review investigated patients with borderline hypoplastic left ventricle who underwent surgical intervention between 2012 and 2022. The patient cohort was stratified into two groups based on the initiation of left ventricle rehabilitation: an early-era group (E group, 2012-2017) and a late-era group (L group, 2018-2022). Left ventricle rehabilitation was defined as palliation combined with other procedures aimed at promoting left ventricular growth such as restriction of atrial septal defect, relief of inflow/outflow obstructive lesions, and resection of endocardial fibroelastosis. A total of 58 patients were included. Primary diagnosis included 12 hypoplastic left heart syndromes, 11 critical aortic valve stenosis, and others. A total of 9 patients underwent left ventricle rehabilitation, 8 of whom underwent restriction of atrial septal defect. As for clinical outcomes, 9 of 23 patients achieved biventricular repair in the E group, whereas in the L group, 27 of 35 patients achieved biventricular repair (39% vs. 77%, p = 0.004). Mortality did not differ statistically between the two groups (log-rank test p = 0.182). As for the changes after left ventricle rehabilitation, left ventricular growth was observed in 8 of 9 patients. The left ventricular end-diastolic volume index (from 11.4 to 30.1 ml/m2, p = 0.017) and left ventricular apex-to-right ventricular apex ratio (from 86 to 106 %, p = 0.014) significantlyincreased after left ventricle rehabilitation. The introduction of the left ventricle rehabilitation strategy resulted in an increased proportion of patients achieving biventricular repair without a concomitant increase in mortality. Left ventricle rehabilitation was associated with enhanced left ventricular growth and the formation of a well-defined left ventricle apex. Our study underscores the significance of left ventricle rehabilitation strategies facilitating successful biventricular repair. The data suggest establishing restrictive atrial communication may be a key factor in promoting left ventricular growth.

Improvised bespoke technique for atrial septostomy in the shortage of atrioseptostomy balloon catheters.

Atrioseptostomy balloon catheter is an essential item to have on our shelves. However, the recall and shortage in production of the commonly used balloon atrioseptostomy catheters posed an imminent threat to our patients. Herein, we present the case of a newborn with a post-natal diagnosis of simple transposition of great arteries and restrictive atrial communication where repeated static balloon atrial septostomy using a 9 mm x 20 mm Sterling balloon failed to improve his status. We had to improvise per-operatively a new bespoke technique to perform a vital pull-through balloon atrial septostomy. The distal third of a 10 mm x 20 mm semi-compliant Cristal balloon was exteriorised out the tip of a 6-Fr 55 cm Cook Flexor sheath in the left atrium, and both were simultaneously pulled back to the right atrium to create an 8 mm septal defect. The procedure was successful without any complications. The baby was weaned off prostaglandin on day 3 and surgically repaired on day 5 with excellent results.

Emergency Use of Occlutech® Atrial Flow Regulator Device Facilitates Weaning From Veno-Arterial Extracorporeal Membrane Oxygenation in a Patient With Severe Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a severe and progressive disease. Treatment options include anti-PAH medications, continuous intravenous therapies, and diuretics. Lung transplant is required in many cases. Atrial septostomy is an under recognized option in symptomatic patients on maximal PAH therapy. However, creating a sustainable and restrictive atrial communication is challenging with existing devices. We describe emergency use of the Occlutech® Atrial Flow Regulator, a novel device, in a 35-year-old female with PAH supported on veno-arterial extracorporeal membrane oxygenation after postpartum decompensation.

Approach to circulation after cardiac surgery in children

Pediatric intensivists are called to patient bedsides in the pediatric cardiac intensive care unit (CICU) after congenital cardiac surgery for low blood pressure (BP) and/or poor perfusion, acute change in heart rate (HR) or rhythm, surgical site bleeding or increased chest tube output, anuria or oliguria, oxygen desaturation less than expected or metabolic acidosis with rising lactic acid and base deficit. Causes of acute circulatory failure after cardiac surgery are divided into four categories which must be considered when approaching the patient in CICU (Table 1).Assessing cardiac output in CICU remains challenging, hemodynamic parameters are usually monitored, along with physical examination, i.e. HR, BP, right and left atrial pressures. There are surrogate markers i.e., mixed venous saturation, brain and renal NIRS, toe temperature, urine output, and then laboratory workup to determine acidosis due to end-organ dysfunction. Echocardiography can confirm low cardiac output syndrome (LCOS) occurs after cardiac surgery with the following major indicators; abnormal ventricular-vascular interaction after bypass, the functionally univentricular circulation, abnormal diastolic function after surgery to the right heart and residual anatomic lesions.1 The limited support tools that are available to manage circulatory failure post cardiac surgery in the CICU are the following: Medications: High labile pulmonary vascular tone (PVR) occurs in patients with pulmonary over-circulation i.e., ASD (atrial septal defect), VSD (ventricular septal defect), PDA (patent ductus arteriosus) and AV canal (atrioventricular canal). Pulmonary venous hypertension, i.e. TAPVR with obstruction, HLHS with restrictive atrial communication and in the univentricular heart after Norwood, shunt or PA band has unstable PVR. Functionally univentricular hearts don't tolerate increased SVR and at the same time, decreased SVR may not be desirable for patients who have fixed systemic or pulmonary obstruction. There are a wide variety of medications to use, but essentially two, milrinone2,3 and epinephrine are very important and widely used. Milrinone is routinely used after cardiac surgery to minimize the LCOS, which works in a receptor independent manner and is synergistic to beta-adrenergic ionotropic effect. Most patients benefit from low dose epinephrine for decreased cardiac function. Nitroprusside is effective where after-load is high, a low dose should always be started titrating to the optimal BP. Generally, there is no role of dopamine in these patients.4 Ventilation-cardiopulmonary interaction: Ventilation at functional residual capacity needs to be targeted. Managing rhythm: Recognition of rhythm is a crucial aspect of care. It is equally important to pay attention to the appropriate heart rate. Extracorporeal mechanical support: The last resort is to put the patient on extracorporeal membrane oxygenation.In summary, the past two decades have seen important advances in our understanding of the circulatory physiology of infants and children post cardiac surgery. When approaching the patients with cardiovascular dysfunction, it is essential to approach the cardiopulmonary system in its entirety, rather than consider the heart, lungs, or peripheral circulation as isolated elements. Working towards one common goal of optimizing systemic oxygen delivery and emphasizing anticipatory intensive care tailored to individual patients with the need for early, targeted investigation and intervention is essential when patients are not progressing as expected.

Restrictive atrial communication in right and left heart failure.

Heart failure (HF) is usually defined by the dominantly affected heart chamber; therefore, termed right or left HF (RHF or LHF). Pulmonologists understand RHF as a complex syndrome characterized by insufficient delivery of blood from the right ventricle associated with elevated systemic venous pressure at rest or exercise. Cardiologists specify LHF by its clinical functional class and the relation to a reduced (HFrEF), preserved (HFpEF) or mid-range ejection fraction (HFmrEF). Pediatric cardiologist, dealing also with patients with a failing single ventricle, define HF as a condition of insufficient systemic oxygen delivery (DO2). Certainly, pediatricians do not think of the right and left heart, or even a single ventricle as an isolated, independently acting entity. Because of the importance of cardiac interactions, the creation of a restrictive atrial communication aims at a palliative approach with the goal to diminish the congestive consequences of a dysfunctional ventricle; further to serve as a pop-off valve in order to prevent syncope and cardiovascular collapse. This review covers the background, the particular indications, the techniques and preliminary results achieved following the creation of a restrictive atrial septum defect (rASD) in different pathophysiological settings. Based on the institutional experience, percutaneous trans-catheter perforation of the atrial septum, followed by gradual balloon dilatation can be performed at any age and location worldwide. Medical institutions in low resource countries can make use of such palliating procedures in the setting of right as well as LHF independent of their pharmacological facilities.

Acute therapy of newborns with critical congenital heart disease.

Critical congenital heart disease (cCHD) is the most common reason for acute cardiac failure in the neonatal period. cCHD, defined by systemic low cardiac output (LCO) and requiring surgery or catheter-based intervention in the first year of life, has an incidence of approximately 15% of CHD and is responsible for up to 25% fatalities of newborn infants. Clinical deterioration develops in most cases due to rapid closure of the ductus arteriosus (DA). Early diagnosis and immediate treatment determinate beneficial outcome. Critical CHD can be classified in duct-dependent systemic flow, duct-dependent pulmonary flow and transposition of the great arteries. The latter two manifest themselves in oxygen resistant cyanosis, whereas CHD with duct-dependent systemic flow may present itself with cardiogenic shock, which can be difficult to differentiate from other causes of shock such as sepsis. Besides prostaglandin therapy for reopening the arterial duct, a balanced parallel pulmonary and systemic circulation should be a therapeutic goal. In CHD with duct-dependent systemic flow a decrease of pulmonary resistance should be avoided; therefore inadequate oxygen therapy, hyperventilation and alkalosis due to excessive treatment of acidosis, should be averted. Volume therapy should be performed carefully. In CHD with duct-dependent pulmonary flow, pulmonary resistance can be decreased, in case of poor pulmonary flow systemic resistance should be increased, mild alkalosis is recommended. Intense volume therapy is in most cases necessary, except if a restrictive atrial communication is present. In addition to intensive care measures, an arsenal of catheter- and surgery-based procedures need to be hold available as back-up for emergency procedures. Transcatheter interventions are nowadays decisive. Atrial-septostomy was the first and still the most utilized high-urgency procedure; DA-stenting is used in prostaglandin-refractory duct stenosis. In the presence of critical aortic valve stenosis, palliation consists of balloon valvuloplasty. In critical aortic coarctation with myocardial failure and no response to prostaglandin, palliative balloon angioplasty may be the method of choice as bridging for corrective surgery.

Creation of a restrictive atrial communication in heart failure with preserved and mid-range ejection fraction: effective palliation of left atrial hypertension and pulmonary congestion.

Left atrial decompression is considered in patients with symptomatic heart failure with preserved ejection fraction (HFpEF). We aimed to evaluate the feasibility and efficacy of transcatheter generation of a restrictive atrial septum communication to manage HFpEF from infancy to adulthood with cardiomyopathy and congenital heart defect. From June 2009 to December 2016, 24 patients (50% with an age less than 16years) with HFpEF were palliated; NYHA-/Ross class IV (n = 10); median systemic ventricular ejection fraction 64 (range 35-78) %. Cardiomyopathy was classified as a restrictive (n = 4) or hypertrophic (n = 2). (75% related to congenital heart defects) Three patients had a systemic right ventricle; in the majority of patients, HFpEF was associated to complex congenital heart defects (n = 18). Mean pulmonary arterial pressures (PAP systolic/diastolic) were 56/28 (± 24/13), left atrial pressures (LAP, v-, a-wave, mean) 26/25/20 (± 7/10/6). Trans-septal puncture was used in 22 patients; foramen ovale dilatation in 2 patients. Median balloon size was 12 (range 6-18) mm; procedure time including diagnostic measures 125 (83-221) min. No procedural death or complications were observed. Mean LA-pressures decreased significantly to 19/19/15 ± 6/8/5mmHg (p = 0.05); median brain natriuretic peptide (BNP) decreased from 392 (range 93-4401) pg/ml median BNP to 314 (range 61-1544) pg/ml (p = 0.05). Three patients died; one patient received orthotopic heart and one patient a heart-lung transplantation. No patient required so far an assist device. Clinical improvement occurred in all patients, in some after additional surgical or interventional approach. Transcatheter LA decompression is an age-independent, effective palliation treating patients with HFpEF.

Creation of a restrictive atrial communication in pulmonary arterial hypertension (PAH): effective palliation of syncope and end-stage heart failure.

Atrial septostomy (AS) is recommended for pulmonary arterial hypertension (PAH)-associated right ventricular (RV) failure, recurrent syncope, or pulmonary hypertensive crisis (PHC). We aimed to evaluate the feasibility and efficacy of AS to manage PAH from infancy to adulthood. From June 2009 to December 2016, transcatheter atrial communications were created in 11 PAH patients (4 girls/women; median age = 4.3 years; range = 33 days–26 years; median body weight = 14 kg; range = 3–71 kg; NYHA-/Ross class IV; n = 11). PAH was classified as idiopathic (n = 6) or secondary (n = 5). History of syncope was dominant (n = 6); two with patent foramen ovale (PFO) admitted with recurrent PHC, three patients required resuscitation before AS. Three patients had PAH-associated low cardiac output. The average pulmonary arterial pressures (PAP systolic/diastolic) were 101/50 (±34/23); the corresponding systemic arterial pressures (SAP) were 99/54 (±23/11); and the mean ratio of PAPd / SAPd was 0.97 (±0.4). Percutaneous trans-septal puncture was uneventfully performed in nine patients; a PFO was dilated in two patients. There was no procedure-related mortality. The median balloon size was 10 mm (range = 6–14 mm); the mean catheter time was 174.6 ± 48 min; fluoroscopy time was 19.8 (±11) min. Syncope and PHC were successfully treated in all patients. The mean arterial oxygen saturation decreased from 97 ± 2 to 89 ± 11.7. One patient died awaiting lung transplantation, one continues to be listed; two patients received a reverse Potts-shunt, one patient died during follow-up; seven patients are stable with PAH-specific treatment. Percutaneous AS is an effective method palliating PAH-associated syncope, PHCs or right (bi-) ventricular heart failure.

Modified Technique to Create Diabolo Stent Configuration.

Diabolo stent configuration aids in stent positioning, stability, and creating a controlled defect with a predetermined size. A number of techniques to create the diabolo configuration have been previously described. The indications for creating a controlled "defect" are rapidly growing and include the Fontan circulation, patients with severe end-stage pulmonary hypertension, restrictive atrial communication in the setting of hypoplastic right or left heart syndrome, and diastolic left heart failure. We describe an alternative technique using a prefabricated readily available tool (gooseneck snare) to create a diabolo stent configuration. The chosen balloon expandable stent is mounted on a 5-mm gooseneck snare centered on an angioplasty catheter larger than 5 mm diameter. When deployed, the snare restricts the central waist to 5 mm and both ends expand to a larger diameter creating a dumbbell/diabolo configuration. A total of six diablo stent configurations were successfully implanted in four patients with failing Fontan physiology; five to create a transcatheter fenestration and one to relieve atrial septal restriction. Data expressed as median and IQR. Their weight was 24.8 kg (19.6-46.95), and age years was 9.2 (6.28-13.23). There were no complications and a consistent diabolo configuration with a 5-mm central waist was created in all patients. The snare serves as a sterile, preconfigured, radiopaque, readily available tool of adequate length and strength, to create consistent diabolo stent configuration without any modifications. This technique is a simple, reproducible, and easy to learn.

Surgical outcome of hypoplastic left heart syndrome with intact atrial septum.

The surgical outcomes of hypoplastic left heart syndrome with intact atrial septum remains very poor in spite of the introduction of prenatal diagnosis before the Norwood operation. The hybrid operation consisting of bilateral pulmonary artery banding and balloon atrioseptectomy is one of the treatment strategies to potentially improve this patient population, however, the long-term outcomes are unknown. Six consecutive patients with hypoplastic left heart syndrome with intact atrial septum, who underwent the hybrid operation between October 2006 and July 2014, were retrospectively reviewed. Hypoplastic left heart syndrome with highly restrictive atrial communication was excluded. Three patients died after the hybrid operation, due to sepsis, cerebral bleeding, and heart failure. Three patients underwent the modified Norwood operation. One of these died due to severe hypoxia while awaiting the bidirectional Glenn operation. The others underwent a bidirectional Glenn operation and subsequent Fontan completion but died due to lung dysfunction and sudden hemoptysis. The hybrid operation for hypoplastic left heart syndrome with intact atrial septum may have improved the neonatal outcome and Fontan completion rate in this extremely high-risk group of patients, but the long-term outcome remains dismal. Considering the late mortality related to pulmonary complications, aggressive fetal intervention to create a nonrestrictive atrial septal communication to promote normal development of the pulmonary vessels may be the last resort to improve the long-term outcome.

Management of hypoplastic left heart syndrome with intact atrial septum: a two-centre experience.

Introduction Hypoplastic left heart syndrome with an intact atrial septum is a poor predictor of outcomes. Prenatal assessment of pulmonary venous Doppler and emergent postnatal cardiac intervention may be associated with better outcomes. Materials and methods A retrospective review of all hypoplastic left heart syndrome patients in two centres over a 5-year period was performed. Group 1 included patients with adequate inter-atrial communication. Group 2 included patients with prenatal diagnosis with an intact atrial septum who had immediate transcatheter intervention. Group 3 included patients with intact atrial septum who were not prenatally diagnosed and underwent either delayed intervention or no intervention before stage 1 palliation. Primary outcome was survival up to stage 2 palliation. The incidence of hypoplastic left heart syndrome with a restrictive atrial communication was 11.2% (n=19 of 170). Overall survival to stage 2 or heart transplantation was 85% and 67% for Groups 1 and 2, respectively (n=129/151, n=8/12; p=0.03), and 0% (n=0/7) for Group 3. Survival benefits were observed between Groups 2 and 3 (p<0.001). Foetal pulmonary vein Doppler reverse/forward velocity time integral ratio of ⩾18% (sensitivity, 0.99, 95% CI, 0.58-1; specificity, 0.99, 95% CI, 0.96-1) was predictive of the need for emergent left atrial decompression. Using a multidisciplinary approach and foetal pulmonary vein Doppler, time-saving measures can be instituted by delivering prenatally diagnosed neonates with hypoplastic left heart syndrome with intact atrial septum close to the cardiac catheterisation suite where left atrial decompression can be performed quickly and safely that may improve survival.

0377: Atrial septostomy in very low birth weight infants

The interventional management of D-Transposition of great arteries DTGA is more required with the advances in NICU and pediatric cardiology. To describe our experience in balloon atrial septestomy (BAS) in D-TGA patients weighing < 1500 g. we reported retrospectively all BAS in VLBW infants from January 2002 to June 2013. We collected information about the vital parameters before and after BAS. We described the atrial septestomy. We have 3 patients with a mean weight of 1333 g. D-TGA was diagnosed at a mean age of 3 days. Four atrial septestomy attempts were done at a mean age of 10.3 days. One infant had two attempts with balloon dilatation in the first, then BAS 24 h later. Procedures were done in catheterization laboratory in three cases and in NICU in one case. Patients were ventilated, sedated and transported by the NICU team. Venous access was obtained by the femoral vein puncture in two cases, femoral vein cutdown in one case and by the umbilical vein in one case. BAS were performed successfully in two cases, one using 4Fr. Swan Ganz catheter, and the other with 5 Fr. Z-Med septestomy catheter. BAS failed in two procedures, one because of atrial wall perforation, and the second because of restrictive foramen ovale which was dilated by a coronary balloon. Complications occurred in 3 cases, one minor (balloon rupture), and two major; atrial perforation in one patient, and severe bradycardia in one patient. BAS was effective with sustained clinical improvement. BAS failure in two patients was followed by heart failure in one patient and the death in the other. BAS is mandatory in VLBW infants with D-TGA and restrictive atrial communication. It should be performed quickly if possible to use the valuable umbilical access, and to avoid the clinical compromise. A holeend BAS catheter is required for successful procedure. FO balloon dilatation constitutes another life-saving option.

728: Maternal hyperoxygenation in the fetus with hypoplastic left heart syndrome (HLHS): A tool for assessment of the pulmonary vasculature

Cardio-pulmonary interactions play an important role in the pathophysiology of HLHS. Pulmonary vasculopathy has been identified at birth, especially in fetuses with a restrictive atrial communication. Responsiveness of the pulmonary vasculature to maternal hyperoxygenation (MH) may provide a tool to assess the degree of fetal pulmonary vasculopathy.

Successful surgical treatment of hypoplastic left heart syndrome associated with a divided left atrium that was diagnosed intraoperatively

Hypoplastic left heart syndrome (HLHS) often involves abnormalities in atrial septal morphology, including a restrictive atrial communication and anomalous attachment of a malaligned septum primum. Cor triatriatum is a rare congenital heart abnormality in which the left atrium (LA) is divided into 2 chambers by a diaphragm. Association of cor triatriatum with HLHS is rare, and cor triatriatum is difficult to diagnose preoperatively. We herein describe an unusual case of HLHS with cor triatriatum.

Aneurysm of the atrial septum in tricuspid atresia: Diagnosis during life and therapy

Massive aneurysmal dilatation of the foramen ovale was diagnosed angiocardiographically in a patient with tricuspid atresia. The angiographic findings are distinct for this condition, and the pathogenesis appears to be related to a restrictive atrial communication in the patient with obligatory right to left shunting at atrial level. The anatomic potential for atrial restriction in the patient with tricuspid or pulmonary atresia, intact ventricular septum and diminutive right ventricle necessitates balloon atrial septostomy at the initial diagnostic cardiac catheterization. In the patient with aneurysmal dilatation of the foramen ovale, satisfactory decompression may be achieved by Blalock-Hanlon atrial septectomy, open atrial septectomy or, possibly, balloon septostomy.