Complex Univentricular Heart Research Articles

Systematic approach to obtain axillary arterial access for pediatric heart catheterizations.

Axillary arterial access (AAA) in pediatric heart catheterizations is undervalued. We retrospectively reviewed children with congenital heart diseases (CHDs) who received trans-axillary arterial catheterizations between January 2019 and February 2023. We aimed ultrasound-guided punctures in the proximal two-thirds of axillary arteries with diameters ≥2 mm to insert 7 cm/4 Fr short introducers. We administrated intra-arterial verapamil (1.25 mg) and heparin (100 UI/kg). We infiltrated per-operatively 2% lignocaine (10 mg) for arterial spasms, long sheaths use (≥5 Fr), and ≥60 min procedures in <3 kg patients. We identified 30 patients (66.7% males) with a median age of 1.1 months (IQR, 0.3-5.4), and a median weight of 3.1 kg (IQR, 2.7-3.7). 5/30 patients had six redo interventions after a median of 3.9 months (IQR, 1.7-5.1). Overall, 27/36 procedures were interventional, including 6 aortic valvuloplasties, 6 balloon angioplasties, and 15 stenting procedures. The median arterial axillary angiographic diameter was 2.6 mm (IQR, 2.4-3). Access was right-sided in 23/36 (63.9%) procedures and obtained using 21G/2.5 cm bevel needles in 25/36 (69.4%) procedures. No hemodynamical change occurred after introducing spasmolytic drugs. The median fluoroscopy time was 26.1 min (IQR, 19.2-34.8). There were two self-resolving arterial dissections, one sub-occlusive arterial thrombosis (resolved with 6 weeks of enoxaparin), and one occlusive arterial thrombosis (resolved with alteplase thrombolysis and 6 weeks of enoxaparin). Median follow-up was 11.7 months (IQR, 8-17.5). Four patients with complex univentricular hearts died from non-procedural causes at a median of 40 days (IQR, 31-161) postoperative. Systematic approach for AAA is the key to success and unlocks the many potentials of trans-axillary pediatric cardiology interventions.

Persisting and reoccurring cyanosis after Fontan operation is associated with increased late mortality.

The Fontan operation aims at reducing cyanosis and cardiac volume overload in patients with complex univentricular heart malformations. However, persisting or reoccurring cyanosis is frequently observed. We sought to systematically determine the prevalence and clinical consequences of persisting and secondary cyanosis after Fontan operation. A total of 331 Fontan patients, operated between 1984 and 2016 with a median postoperative follow-up of 7.9 (interquartile range 2.6-15.8) years, were studied retrospectively. Cyanosis was defined as transcutaneous oxygen saturation ≤93% at rest measured by pulse oximetry. Prevalence of cyanosis was analysed at 3 different time points (t1 = post-Fontan operation, t2 = post-Fontan cardiac catheterization, t3 = last follow-up) and the association of cyanosis with mortality was examined. Prevalence of cyanosis was 50% at t1 and 39% at t3. Fenestration was patent in 71% and 33% of all cyanotic patients at t1 and t3, respectively. In patients with clinical indication for catheterization (t2; n = 178/331), prevalence of cyanosis was 72%. At t2, patent fenestration (33%), veno-venous collaterals (24%) or both (32%) were present. Thirty-six (11%) patients died during follow-up. In a time-varying multivariable Cox regression analysis, cyanosis was the strongest predictor for late mortality (P < 0.001, hazard ratio 12.2, 95% confidence interval 3.7-40.5). Prevalence of cyanosis was considerable during long-term follow-up after Fontan operation and-as a surrogate parameter for unfavourable Fontan haemodynamics-is associated with increased late mortality. Accordingly, particular attention should be directed towards the persistence or reoccurrence of cyanosis during follow-up since it may indicate haemodynamic attrition and development of Fontan failure.

Five decades of the Fontan operation: A systematic review of international reports on outcomes after univentricular palliation.

Almost fifty years after its first clinical application, the modified Fontan operation is among the most frequently performed procedures in congenital heart disease surgery in children today. The objective of this review is to systematically summarize the international evolution of outcomes in regard to morbidity and mortality of patients with Fontan palliation. All studies published over the past five decades with more than 100 Fontan patients included were screened. In eligible studies, information concerning preoperative patients' characteristics, Fontan modifications employed, early mortality, long-term survival and frequency of relevant complications was extracted. Ultimately, thirty-one studies published by the largest surgical centers with an overall number of 9390 patients were included in this review. The extracardiac total cavopulmonary anastomosis is the most frequently used Fontan modification. Hemodynamic data demonstrate a rigorous overall adherence to suggested Fontan selection criteria. The analysis showed a clear trend toward improved early and long-term survival over the time period covered. Although inconsistently reported, severe complications such as arrhythmias, thromboembolic events and protein-losing enteropathy as well as reoperations and reinterventions were frequent. In conclusion, patients palliated for complex univentricular heart malformations nowadays benefit from the experience and technical developments of the past decades and have a significantly improved long-term prognosis. However, important issues concerning postoperative long-term morbidity and mortality are still unsolved and clear intrinsic limitations of the Fontan circulation are becoming evident as the population of Fontan patients ages.

Late complications of Fontan operation

Complex univentricular heart defects are treated with the Fontan operation that consists in the diversion of the systemic venous return to the pulmonary arteries. Currently, the operation is achieved through a staged approach and with the use of prosthetic extracardiac conduits to connect the inferior vena cava to the pulmonary arteries. The improvement of early and late survival allowed these patients to reach adulthood, although long-term complications remain a relevant problem. Most of the complications are caused by chronic systemic venous hypertension due to the lack of a pumping sub-pulmonary ventricle. The most frequent complications are arrhythmias, thromboembolism, protein-losing enteropathy, heart failure and plastic bronchitis. The pathogenetic mechanisms are not completely understood but they are all connected to chronic systemic venous hypertension. Quality of life and life expectancy are deeply impaired by the onset of such complications and then a strict follow-up is required to recognize early sings of complications and to start early treatment. The creation of a highly specialized referral center network for the treatment of such complications and delicate patients is needed to improve the efficacy and the outcomes for the Fontan patients.

Direct Left Atrial Pressure Measurement with Pressure Sensing Wires in Complex Univentricular Heart Disease

Direct Left Atrial Pressure Measurement with Pressure Sensing Wires in Complex Univentricular Heart Disease

Modified Aortic Uncrossing Procedure: A Novel Approach for Norwood Palliation of Complex Univentricular Congenital Heart Disease With a Circumflex Aorta

The circumflex aorta is a rare type of true vascular ring anomaly. It consists of a retroesophageal right aortic arch, a left-sided descending thoracic aorta, and a left-sided ligamentum arteriosum. The "aortic uncrossing procedure" described by Planché and Lacour-Gayet is the procedure of choice for managing this aortic anomaly in patients with a biventricular heart. The presence of a circumflex aorta in a patient with heterotaxy syndrome and univentricular congenital heart disease requiring Norwood palliation is highly unusual. We report such a case and describe our approach to its surgical management.

Clinical application of three-dimensional printing to the management of complex univentricular hearts with abnormal systemic or pulmonary venous drainage.

In recent years, three-dimensional printing has demonstrated reliable reproducibility of several organs including hearts with complex congenital cardiac anomalies. This represents the next step in advanced image processing and can be used to plan surgical repair. In this study, we describe three children with complex univentricular hearts and abnormal systemic or pulmonary venous drainage, in whom three-dimensional printed models based on CT data assisted with preoperative planning. For two children, after group discussion and examination of the models, a decision was made not to proceed with surgery. We extend the current clinical experience with three-dimensional printed modelling and discuss the benefits of such models in the setting of managing complex surgical problems in children with univentricular circulation and abnormal systemic or pulmonary venous drainage.

The blalock and taussig shunt revisited.

The systemic to pulmonary artery shunts are done as palliative procedures for cyanotic congenital heart diseases ranging from simple tetralogy of Fallots (TOFs)/pulmonary atresia (PA) to complex univentricular hearts. They allow growth of pulmonary arteries and maintain regulated blood flow to the lungs till a proper age and body weight suitable for definitive corrective repair is reached. We have reviewed the BT shunt with its anaesthtic considerations and management of associated complications.

Hereditary Thrombophilic Disorders to Not Contribute to the Risk of Thrombotic and Thromboembolic Events in Patients with Complex Univentricular Heart Malformations

Hereditary Thrombophilic Disorders to Not Contribute to the Risk of Thrombotic and Thromboembolic Events in Patients with Complex Univentricular Heart Malformations

Current outcomes of the bi-directional cavopulmonary anastomosis in single ventricle patients: analysis of risk factors for morbidity and mortality, and suitability for Fontan completion.

The bi-directional cavopulmonary anastomosis forms an essential staging procedure for univentricular hearts. This review aims to identify risk factors for morbidity, mortality, and suitability for Fontan completion. A total of 114 patients undergoing cavopulmonary anastomosis between 1992 and 2012 were reviewed to assess primary - mortality and survival to Fontan completion - and secondary outcome endpoints - re-intubation, new drain, and ICU stay. Median age and weight were 8 months and 6.9 kg, respectively. In 83% of patients, 1-3 interventions had preceded. Norwood-type procedures became more prevalent over time. Extubation occurred after a median of 4 hours, median ICU stay was 2 days; 10 patients (8.8%) needed re-intubation and 18 received a new drain. Higher central venous pressure and transpulmonary gradient were risk factors for new drain insertion (p<0.01). Higher pre-operative pulmonary pressure correlated with increased inotropic support and prolonged intubation (p=0.01). Need for re-intubation was significantly affected by younger age at operation (p=0.01). Hospital and pre-Fontan mortality were 11.4 and 5.3%, respectively. Operative mortality was independently affected by younger age (p=0.013), lower weight (p=0.02), longer bypass time (p=0.04), and re-intubation (p=0.004). Interstage mortality was mainly influenced by moderate ventricular function (p=0.03); 82% of survivors underwent or are candidates for Fontan completion. The cavopulmonary anastomosis remains associated with adverse outcomes. Age at operation decreases with rising prevalence of complex univentricular hearts. Considering the important impact of re-intubation on hospital mortality, peri-operative management should focus on optimising cardio-respiratory status. Once this selection step is taken, successful Fontan completion can be expected, provided that ventricular function is maintained.

Aortic arch and pulmonary artery reconstruction during heart transplantation after failed Fontan procedure: Figure 1:

Recent significant improvement in surgical management of patients with univentricular palliations has resulted in an increasing number of patients with complex univentricular heart repairs surviving to heart transplantation. Heart transplantation in these patients can be challenging and may require extensive vascular reconstruction during transplantation. Herein, we describe the surgical reconstruction in a complex patient that was required prior to heart transplantation.

Comprehensive dental evaluation of children with congenital or acquired heart disease

The aims of this case–control study were to (a) compare the caries experience and oral hygiene, and (b) quantify the persistence of a delay in the dental age in children with cardiac disease and a group of healthy children. The study population comprised a group of 268 3- to 16-year-old children and adolescents with a cardiac disease and a group of 268 age- and sex-matched healthy children and adolescents. Specifically, the decayed, missed, and filled teeth indices, simplified oral hygiene index, and the dental ages of the two groups of children were calculated and then compared. Although the oral health of the children with either a congenital or an acquired heart disease was the same as that of the healthy children, there were significant differences in the decayed, missed, and filled teeth indices. Dental ages of the children with a congenital heart disease were significantly lower than those of healthy children. The findings showed that complex univentricular heart diseases had the highest negative impact on dental development (21.1), followed by complex biventricular (20.9), simple surgical (20.5), and mild (20.4) heart disease patients. Once thorough knowledge of the child’s cardiac status is gained, a definitive dental treatment plan for the child with a cardiac disease can be established.

A flow restrictor implanted percutaneously across a loose pulmonary artery band

Pulmonary artery banding usually is performed as a palliative procedure. In patients with elevated pulmonary vascular resistance and complex congenital heart disease, it may not be possible at the time of surgery to obtain sufficient restriction to optimize the patient for further treatment; additional restriction may be needed in time. We present a technique where we used a combination of two devices (a 10-mm Amplatzer ASD occluder fenestrated with a Palmaz Genesis 9/19 mm stent) to percutaneously further reduce the flow to the lungs 1 month after surgical placement of a pulmonary artery band in a 16-year-old girl with complex univentricular heart. Eight months after the banding the patient successfully underwent completion of the Fontan circulation by total cavopulmonary connection. This technique allows to reduce flow without redo surgery. In well-selected patients, this new percutaneous technique allows to better prepare such complex patients for future surgery.

Origins of Neonatal Heart Transplantation: An Historical Perspective

Clinical heart transplantation was first accomplished by Christiaan Barnard in 1967. Infant heart transplantation was first attempted by Adrian Kantrowitz just 3 days later in New York. Sixteen years lapsed before neonatal transplantation was again attempted, first in London and then in Loma Linda, California in the summer and fall of 1984, respectively. The latter infant became known as "Baby Fae." Neither infant survived a month, but both produced an important impact through media exposure on the public consciousness. The idea of infant organ donation slowly developed traction. The first successful neonatal heart transplant occurred in Loma Linda on November 15, 1985. That recipient, born with hypoplastic left heart syndrome, is now 25 years old. Thereafter, the incidence of infant and pediatric heart transplantation gradually increased. Today, 350 to 450 transplants are reported annually to the Registry of the International Society for Heart and Lung Transplantation, 25% of which are in young infants. Norwood's staged-reconstruction for complex univentricular heart disease has markedly reduced the need for primary transplantation in very early life. Still, many potential young infant recipients are best treated in no other way.

Percutaneous Device Closure of a Bifurcated Inferior Vena Cava and Completion of the Kawashima Procedure for the “Difficult” Univentricular Heart

Azygos continuation of inferior venacava (IVC) is well known in association with interrupted IVC. We report azygos connection of uninterrupted IVC in a young child with complex univentricular heart. This peculiar anatomy was made suitable for univentricular repair by combining percutaneous device closure of prehepatic right limb of IVC and bidirectional Glenn shunt. The azygos vein acted as a conduit for IVC flow to superior venacava as in Kawashima's operation. This case highlights a rare IVC morphology. Also this innovative strategy allowed simple solution to a challenging problem and is first time described in the literature.

Evolution of the Fontan-Kreutzer Procedure

The surgical techniques used to construct the Fontan-Kreutzer circulation have evolved considerably since the initial reports. In the early years, it was thought that a power source other than the main ventricular chamber was useful or even necessary to serve the pulmonary circulation. Better understanding of the physiology of the Fontan-Kreutzer circulation has led to an understanding that avoidance of flow disturbances and energy losses in the surgically constructed pathways is critically important. In vitro studies and clinical investigations of flow dynamics led to the introduction of the total cavopulmonary connection, or total cavopulmonary connections, designed to minimize flow disturbances and the resultant energy losses. Other important surgical modifications include staging with a superior cavopulmonary connection and creation of a fenestration. These innovations have resulted in extension of the Fontan-Kreutzer procedure to the management of complex univentricular hearts and, in particular, the management of hypoplastic left heart syndrome. There have been significant improvements of early mortality and morbidity following the Fontan-Kreutzer procedure. Yet it is important to recognize that there is continuing late attrition and morbidity for patients with the Fontan-Kreutzer circulation.

Three hundred and thirty-three experiences with the bidirectional Glenn procedure in a single institute

Introduction of the bidirectional Glenn procedure (BDG) in low-risk Fontan candidates would improve clinical outcomes. Over the last decade, not only high-risk Fontan candidates, but all candidates underwent BDG and staged Fontan operation (TCPC) in our hospital. Three hundred and thirty-three consecutive patients (age range, 42 days to 16 years old) underwent BDG at Fukuoka Children's Hospital Medical Center from 1992 to 2004. Diagnoses included hypoplastic left heart syndrome in 47, pulmonary atresia with intact ventricular septum in 32, tricuspid valve atresia in 35, and other complex univentricular heart defects in 219 patients (right dominant in 166, left dominant in 53). There were three hospital deaths and 27 late deaths (five after TCPC). Six patients underwent takedown operation. Two hundred and thirty patients underwent TCPC, while 66 patients were waiting for TCPC. In five patients, completion of TCPC was contraindicated. A univariate analysis revealed that for patients less than six months old, diagnoses besides tricuspid atresia, right ventricular morphology, mean pulmonary arterial pressure, pulmonary vascular resistance, ventricular end-diastolic pressure, atrioventricular valve regurgitation greater than moderate, atrioventricular valvuloplasty/valve replacement in concomitant procedure, and total anomalous pulmonary venous connection repair in concomitant procedure were significant predictors of death, takedown, or out of indication for completion of TCPC. A stepwise logistic regression analysis showed that mean pulmonary arterial pressure and heterotaxy were independent predictors. The staged strategy used for all Fontan candidates provides excellent clinical results. The main risk factor for death, takedown, or out of indication for completion of TCPC was elevated pulmonary arterial pressure. Appropriate surgical interventions such as atrioventricular valvuloplasty and total anomalous pulmonary venous connection repair, before and/or on BDG for the control of pulmonary circulation are of great importance to prevent elevation of pulmonary arterial pressure.

Fontan modification for subsequent non-surgical Fontan completion.

Establishment of Fontan circulation in complex univentricular hearts often requires several surgical procedures. We developed a procedure which maintains the advantages of a staged approach, however, during the initial surgery additional preparatory measures are performed to allow subsequent non-surgical Fontan completion. The operation is a lateral baffle Fontan procedure. The baffle bears multiple perforations to allow the inferior vena cava blood to drain into the systemic atrium. Total cavopulmonary connection is performed as usual and the cardiac end of the superior vena cava is subtotally banded. Formally the operation establishes a bi-directional Glenn physiology. During subsequent catheter intervention the banding of the superior vena cava is dilated and the holes in the baffle are closed with appropriate devices. From April 1994 to December 1995, 18 children having at least two risk factors for Fontan operation received the above described operation. Ages ranged from 3 months to 15 years. Ten patients had one or more previous operations. Bypass time ranged from 86 to 128 min and cross clamp time from 14 to 79 min. O2 saturation after discontinuation of cardiopulmonary bypass was 76% (70-81%). The postoperative recovery of all patients was rapid with early extubation (mean 6 h) and discharge to the ward the morning of the first postoperative day. One patient died. No fluid retention as pericardial, pleural or abdominal fluid effusions occurred. At discharge O2 saturation was 77% (75-82%). In thirteen children successful conversion to total cavopulmonary connection with interventional debanding of the superior vena cava and closure of the fenestrations was performed. After a hospital stay of only a couple of days the children were discharged with normal O2 saturation after Fontan completion. We suggest that this modification of the staged Fontan procedure reduces the need for surgical interventions by applying balloon angioplasty and occluder technology to this unique subset of patients.

Total cavopulmonary anastomosis in patients less than three years of age

As young age at modified Fontan operation was thought to be a preoperative risk factor for poorer survival, we studied early and intermediate outcome in our young patient group. Results in children less than 3 years of age (group I; n = 26; age range, 7 to 35 months) were compared with those in older patients (group II; n = 46; age range, 36 to 219 months). For both groups there was no significant difference with regard to preoperative pathology and hemodynamics. With an overall mortality of 9.7% (7/72) there was no significant difference for both groups. Group I (n = 23) and group II (n = 42) survivors did not differ with respect to early postoperative incidence of atrial dysrhythmias, duration and volume of pleural effusion, or incidence of reoperation. Results on intermediate follow-up (group I, 31 +/- 14 months; group II, 44 +/- 20 months) demonstrated a relatively low incidence of late atrial dysrhythmias (12.3%; 8/65). Late atrioventricular valve insufficiency was significantly more frequent in group I patients. With similar preoperative anatomic and hemodynamic parameters including 68% of patients with complex univentricular heart, total cavopulmonary anastomosis could be performed in patients less than 3 years of age with good clinical and hemodynamic results, as achieved in older patients.

Damus-Stansel-Kaye procedure: Current indications and results

Between October 1983 and August 1991, 29 consecutive Damus-Stansel-Kaye procedures were performed. Indications for operation included restrictive bulboventricular foramen or subaortic stenosis associated with complex univentricular congenital heart disease (25) and Taussig-Bing heart, subaortic stenosis, or both associated with complex biventricular congenital heart disease (4). Twelve patients underwent concurrent Fontan procedures. Average age at operation was 39.8 months (range, 1 to 132 months). Average outflow tract gradient was 28 mm Hg (range, dynamic to 80 mm Hg). Of the 29 patients, 23 were male and 6 were female. There were three early deaths (10%), two in patients who had a concurrent Fontan procedure. Although there was a trend toward lower age and higher outflow tract gradients in nonsurvivors, these and other factors were not statistically significant predictors of death. Actuarial freedom from cardiac-related death was 88%. at 5 years (n = 7). In a mean follow-up of 3.5 years (range, 0.1 to 7.7 years), 3 patients have required reoperation (10%), 2 for aortic valve insufficiency (5 days and 2.75 years) and 1 for a gradient across the anastomosis (5.75 years). Actuarial freedom from reoperation related to a failed Damus-Stansel-Kaye procedure was 90% at 4 years and 75% at 6 years (n = 7).