Anatomy of the Functionally Univentricular Heart

2018 ◽  
Vol 9 (6) ◽  
pp. 677-684 ◽  
Author(s):  
Robert H. Anderson ◽  
Rodney C. G. Franklin ◽  
Diane E. Spicer
Keyword(s):  
Cardiac Surgery ◽  
Left Ventricle ◽  
Right Ventricle ◽  
Congenital Heart ◽  
Operative Procedure ◽  
Fontan Circulation ◽  
Pediatric Cardiac Surgery ◽  
Univentricular Heart ◽  
Ventricular Mass ◽  
The World

The world of pediatric cardiac surgery and cardiac surgery as a whole lost one of the great pioneers with the passing, at the beginning of 2018, of Francis Fontan. Hence to add to the recognition of his achievements, the European Congenital Heart Surgeons Association (ECHSA) has established a lecture to be given in his memory at their annual meetings. It was a significant honor and privilege to be invited to present the initial lecture. In this report, we describe the essence of the presentation. Many patients are now palliated by construction of the Fontan circulation. Very few of those put forward for this operative procedure have anatomically univentricular hearts. It remains frequent, nonetheless, to find accounts of many patients allegedly having “single” ventricles. We discuss the background to this illogical approach to description of hearts having one big and one small ventricle, showing that those with normal hearts have a single left ventricle, albeit co-existing with a single right ventricle. We show that analysis of the ventricular mass in tripartite fashion produces much needed clarity in the appropriate description of the ventricular mass in those increasingly submitted for construction of the Fontan circulation. We emphasize that although it was patients with univentricular atrioventricular connections who were the first to benefit from the procedure, the majority of patients now have biventricular atrioventricular connections, although the hypoplastic ventricle possesses all three of its normal components. We show that description of the ventricular arrangement as being functionally, or physiologically, univentricular provides logic in what had previously been an illogical environment.

Morphology of the functionally univentricular heart

2004 ◽  
Vol 14 (S1) ◽  
pp. 3-12 ◽  
Author(s):  
Robert H. Anderson ◽  
Andrew C. Cook
Keyword(s):  
Left Ventricle ◽  
Right Ventricle ◽  
Univentricular Heart ◽  
Separate Analysis ◽  
Logical Description ◽  
Morphological Method ◽  
Ventricular Mass ◽  
Anatomical Arrangement ◽  
Mass Functions ◽  
Double Inlet Left Ventricle

Of all the complex malformations that affect the heart, those that have produced the greatest difficulty, over the years, in terms of logical description and classification are the ones with the circulations supported by a functionally univentricular mass. The problems in description stem from the fact that, although the ventricular mass functions as a singular entity, the majority of lesions falling within this category, from the stance of morphology, possess two ventricular chambers. For many years, and indeed, even now in many centres, the hearts are described as being “univentricular”, or alternatively as “single ventricles”. There is still no consensus as to which particular lesions should be included within this “univentricular” category. The paradigm of the group is usually taken as double inlet left ventricle.1In this lesion, the dominant left ventricle is accompanied by a rudimentary second chamber, albeit that arguments continue as to whether the second chamber is an incomplete right ventricle,2or simply an infundibulum.3Further arguments raged as to whether tricuspid atresia should be included within the univentricular category.4,5The debate is rationalised when we recognise that all the potentially univentricular hearts are treated nowadays by constructing the Fontan circulation,6or one of its variants,7as indeed are some unequivocally biventricular hearts.8Clarification and simplification of the anatomical arrangement in this functionally univentricular category, as we will show in this review, is provided by rigorous application of the crucial philosophic principle of analysis called the “Morphological Method”,9coupled with separate analysis of the ventricular mass and the atrioventricular junctions.10,11

scholarly journals Incidence and Outcomes of Patients with Functionally Univentricular Heart Born in Latvia, 2007 to 2015

Medicina ◽  
2018 ◽  
Vol 54 (3) ◽  
pp. 44
Author(s):  
Katrina Rutka ◽  
Inguna Lubaua ◽  
Elina Ligere ◽  
Amanda Smildzere ◽  
Valts Ozolins ◽  
...  
Keyword(s):  
Cardiac Surgery ◽  
Left Ventricle ◽  
Right Ventricle ◽  
High Mortality ◽  
Heart Defects ◽  
University Hospital ◽  
Univentricular Heart ◽  
Hypoplastic Left Ventricle ◽  
Hypoplastic Right Ventricle ◽  
Surgery Center

Background and Objectives: A functionally univentricular heart is the term used to describe congenital heart defects where it is impossible to restore two pumping chambers. These lesions are associated with high mortality, morbidity, and medical resource utilization. The aim of this study was to review incidence and outcomes of patients with a functionally univentricular heart at the only pediatric cardiac surgery center in Latvia. Methods: We performed a retrospective review of medical records of (i) all children with a functionally univentricular heart treated at the Clinic of Pediatric Cardiology and Cardiac Surgery, and (ii) all prenatally diagnosed cases of univentricular heart at Children’s Clinical University Hospital in Latvia. We reviewed data regarding children born from January 1, 2007, to December 31, 2015. The children’s cardiac anatomy and interventions were categorized in accordance with the International Pediatric and Congenital Cardiac Code (v3.3). Results: During the study period, 49 patients with a functionally univentricular heart were admitted to Children’s Clinical University Hospital with a corrected incidence of 0.69 per 1000 live births per year. There were 26 patients that had a hypoplastic left ventricle, and 22 patients that had a hypoplastic right ventricle, while one patient had an indeterminate ventricle. Thirty (61.2%) patients had died by the end of data collection. Twenty-one of the 30 deaths occurred before or immediately after stage I surgical palliation. Cumulative neonatal and 5-year survival of patients with a hypoplastic right ventricle was 81.8% and 63.6%, respectively; for patients with hypoplastic left ventricle—46.2% and 17.3%, respectively. Discussion: This is the first mid-term outcome study of patients with a univentricular heart in Latvia. The high mortality reflects the challenges of a small-volume, developing congenital cardiac surgery center. Data from this study will be used as a baseline for quality improvement.

Thromboelastrography Reduces Blood Product Transfusion in Pediatric Cardiac Surgery

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1176-1176
Author(s):  
Michael Ritchie ◽  
Cathy Woodward ◽  
Lauren Kane ◽  
Melissa Frei-Jones
Keyword(s):  
Cardiac Surgery ◽  
Congenital Heart ◽  
Blood Product ◽  
Pediatric Cardiac Surgery ◽  
Blood Product Transfusion ◽  
Red Cell ◽  
Time Period ◽  
Significant Difference ◽  
Operative Complications ◽  
Post Operative Complications

Abstract Abstract 1176 Thromboelastography (TEG) has emerged as an important tool to guide blood product transfusions in pediatric cardiac surgery requiring cardio-pulmonary bypass (CPB). Blood product transfusions are associated with risk including transfusion transmitted infections, transfusion reactions, and allo-immunization. Previous studies have reported fewer red cell and plasma transfusions but increased platelet transfusions with no difference in post-operative bleeding in pediatric CPB using TEG to determine transfusion needs. In this study, we evaluated the use of intra-operative TEG to reduce blood product transfusion in pediatric cardiac surgery with CPB. A retrospective case control study of 150 patients, age birth to 18 years, who required CPB during cardiac surgery, was performed from January 1, 2010 to May 31, 2012. Cases were chosen serially during the time period when TEG was utilized by anesthesia. Controls were chosen from the time period before TEG was available. Exclusion criteria were a personal or family history of bleeding or clotting disorder. Controls were matched 2:1 on age and Risk Adjustment for Congenital Heart Surgery score (RACHS). The type and amount of blood product transfusions were compared between cases and controls in addition to post-operative complications including bleeding, infection and thrombosis. This study included 50 cases and 100 controls. Average age and gender were not different between cases and controls (19 mo (0–213) vs 20 mo (0–255), p=0.86; 52% (26/50) males vs 62% (62/100), p=0.24). Ethnicity was similar between groups and primarily Hispanic (66% (33/50) vs 70% (70/100), p=0.71). The most common congenital heart defect was Tetralogy of Fallot (20% (10/50) vs 22% (22/100); p=0.84). The median RACHS score between groups was the same (3 (2–6) vs 3 (2–6), p=0.88). There was no significant difference in pre-surgical or post-surgical blood counts, coagulation testing or CPB pump time. The average number of TEGs performed per case was 2.6 (1–6). Cases received significantly fewer platelet and cryoprecipitate (cryo) units but similar red cell and plasma units to controls as shown in Figure 1. The difference persisted when transfusions were adjusted for weight. Cases received fewer platelets (13 (0–49) ml/kg vs 21 (0–119) ml/kg, p=0.015), and cryo (3 (0–36) ml/kg vs 6.3 (0–47) ml/kg, p=0.029) with the most significant difference seen in patients less than 10 kg (platelets 15 ml/kg vs 25 ml/kg, p=0.007; cryo 4 ml/kg vs 8 ml/kg, p=0.03). There was no difference in red cell volume (130 (0–332) ml/kg vs 133 (0–680) ml/kg, p=0.88), or plasma volume (109 (0–277) ml/kg vs 107 (0–553) ml/kg, p=0.9) at any weight between groups. There was no statistical difference in PICU length of stay (LOS), hospital LOS, mechanical ventilation, survival to discharge or frequency of post-operative bleeding or thrombosis. There was a 50% reduction in hospital cost of platelet transfusions ($29,750 vs $65,450) and cryo ($1,950 vs $4,700) for the 50 cases compared to controls. The cost of three TEGs per 50 cases was $3,450 ($23/TEG) for a total cost savings of $35,000. Intra-operative TEG reduced the amount of platelet and cryoprecipitate transfusions used during pediatric CPB without an increase in post-operative complications. The reduction in blood product administration by using TEG resulted in decreased cost. Disclosures: No relevant conflicts of interest to declare.

Pediatric cardiac surgery in Indonesia

1998 ◽  
Vol 8 (4) ◽  
pp. 437-439 ◽  
Author(s):  
Toshihide Asou ◽  
Jusuf Rachmat
Keyword(s):  
Congenital Heart Disease ◽  
Cardiac Surgery ◽  
Heart Disease ◽  
Surgical Outcome ◽  
Congenital Heart ◽  
Pediatric Cardiac Surgery ◽  
Cardiac Surgeons ◽  
Cardiac Center ◽  
Doctors And Nurses ◽  
At Home

AbstractPediatric cardiac surgery in Indonesia first developed thanks to the cooperation of various cardiac centers abroad. The establishment of the ‘Harapan Kita’ National Cardiac Center in 1985 was one of the most important initial steps. Thereafter, the discipline advanced remarkably in terms of the number of the operations performed and the variety of the diseases treated and, as a result, the surgical outcome also improved. Numerous problems remain to be solved. Only 1% of the children with congenital heart disease are today properly treated in Indonesia. Some of the underlying problems responsible for this situation include a shortage of pediatric cardiac professionals, the lack of the information and education on the part of the patients, and a shortage of funding, both privately and publicly. It would thus be welcome for pediatric cardiac surgeons, cardiologists and nurses in Indonesia to learn about congenital heart disease from doctors and nurses in advanced countries in order to improve the outlook at home.

scholarly journals 97 An unusual combination of rare congenital heart defects in a 70 years old lady complaining worsening dyspnea

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Matteo Bellettini ◽  
Antonio Montefusco ◽  
Andrea Angelini ◽  
Fulvio Orzan ◽  
Fabrizio D’Ascenzo ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Left Ventricle ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Tricuspid Regurgitation ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Pulmonary Regurgitation ◽  
Subaortic Membrane ◽  
The Right

Abstract Methods and results A 70-year-old woman presented to our outpatient clinic complaining of worsening dyspnoea in the last 3 months. She had a medical history of hypertension, diabetes, dyslipidemia, and paroxysmal atrial fibrillation. We performed a comprehensive evaluation starting with a transthoracic echocardiogram that showed a dilatation of right ventricle with normal function, severe pulmonary regurgitation, and moderate tricuspid regurgitation with estimated pulmonary artery systolic pressure of 55 mmHg; the left ventricle had normal dimension and function, with mild aortic and mitral regurgitation, and a subaortic membrane which caused a mild obstruction (maximum gradient 17 mmHg). The cardiac magnetic resonance (CMR) confirmed the enlargement of the right ventricle and of the pulmonary artery trunk (51 mm) and the severity of pulmonary regurgitation (regurgitant fraction of 41%). CMR also clearly showed the VSD just below the subaortic membrane and the left to right shunt with a jet that appeared to proceed straight from the left ventricle through the pulmonary valve (Figure 1A). The estimated Qp/Qs was 1.6 and no intramyocardial late enhancement was present. Pulmonary pressures and pulmonary vascular resistance were normal at the right heart catheterization and the Qp/Qs ratio calculated invasively was 1.45. Considering patient high-risk profile for coronary artery disease, a coronary angiography was also performed showing an abnormal origin of the right coronary artery (RCA) from the mid-portion of the left anterior descending coronary artery (LAD) with two significant stenosis: one involving the bifurcation of RCA and the other the mid-portion of the LAD (Figure 1B). The coronary computed tomography angiography (CCTA) showed a benign course of the RCA anterior to the pulmonary artery towards the auriculoventricular groove (Figure 1C, D). Taking into account all these findings, multidisciplinary heart team decided to perform a cardiac surgery intervention of pulmonary valve and trunk replacement, closure of ventricular septal defect and two coronary bypass grafts on LAD and RCA. Conclusions This case represents a combination of some rare congenital heart abnormalities where multimodality cardiovascular imaging techniques were essential to establish a proper diagnosis and to plan an adequate surgical repair. We hypothesize that the peculiar orientation of the VSD jet may have caused the pulmonary trunk dilatation considering that neither the shunt, nor the pulmonary pressure appear to have been of sufficient magnitude to cause it. Pulmonary ectasia and the damage inflicted by the jet to the cusps of the valve have led to the severe valvular insufficiency. While aortic and tricuspid regurgitation are known to be associated with VSD, to the best of our knowledge this is the first report of pulmonary regurgitation secondary to VSD.

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