scholarly journals The effect of hypocapnia on systemic perfusion in patients with single ventricle after surgery

2021 ◽  
Vol 18 (1) ◽  
pp. 65-74
Author(s):  
A. В. Naumov ◽  
G. G. Khubulava ◽  
Yu. S. Аleksandrovich ◽  
S. P. Marchenko ◽  
К. V. Pshenisnov ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Blood Flow ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Defects ◽  
Venous Blood ◽  
Extraction Ratio ◽  
Mixed Venous Blood ◽  
O2 Extraction ◽  
Mixed Venous

The objective: the aim of the study was to identify the relationship between arterial hypocapnia and systemic hypoperfusion in newborns with single ventricular physiology after hemodynamic correction of congenital heart disease. Subjects and methods. 125 newborns with congenital heart defects operated from 2014 to 2018 were examined retrospectively.  Arterial and central venous blood gases were collected in the postoperative period.  A total of 670 pairs of laboratory results were selected.Results. Based on the presence/absence of hypocapnia (PaCO2 less than 35 mm Hg), 2 groups were formed. Group G-0 (the hypocapnic variant of the single-ventricular circulation) comprised 44 observations. Group G-1 (PaCO2 more than 35 mm Hg) included 40 observations.  In 32 (38%) cases the level of systemic perfusion was within the normal range, in 52 (62%) cases, systemic hypoperfusion was detected.  In samples corresponding to Group G-1, signs of DOS were observed in 20 cases.  The study showed that the most pronounced intergroup difference in parametric data was observed among indicators reflecting oxygen consumption and, as a consequence, the system flow rate (РO2 in mixed venous blood, saturation in mixed venous blood, arterio-venous difference in saturation, O2 content in venous blood, O2 extraction ratio, arterio-venous difference in РCO2).  In addition, the HF markers such as arterio-venous difference in saturation, O2 extraction ratio, arterio-venous difference in РCO2 had a strong correlation with the signs of systemic hypoperfusion. In the hypocapnic group, the tendency for more pronounced desaturation of venous blood was determined, and a higher arterio-venous difference in saturation, O2 content in venous blood, O2 extraction ratio, and arterio-venous difference in РCO2 parameters were also noted.Conclusions. Arterial hypocapnia may be a sign of pulmonary overflow and reduction of systemic blood flow in newborns with single ventricular physiology, after hemodynamic correction of congenital heart disease.  When managing newborns with parallel circulation, hypocapnia should be avoided as a factor contributing to the redistribution of blood flow from left to right and the development of systemic hypoperfusion. 

scholarly journals Fetal Blood Flow and Genetic Mutations in Conotruncal Congenital Heart Disease

2021 ◽  
Vol 8 (8) ◽  
pp. 90
Author(s):  
Laura A. Dyer ◽  
Sandra Rugonyi
Keyword(s):  
Congenital Heart Disease ◽  
Blood Flow ◽  
Heart Disease ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Heart Defects ◽  
Genetic Mutations ◽  
Fetal Blood ◽  
Persistent Truncus Arteriosus ◽  
Fetal Circulation

In congenital heart disease, the presence of structural defects affects blood flow in the heart and circulation. However, because the fetal circulation bypasses the lungs, fetuses with cyanotic heart defects can survive in utero but need prompt intervention to survive after birth. Tetralogy of Fallot and persistent truncus arteriosus are two of the most significant conotruncal heart defects. In both defects, blood access to the lungs is restricted or non-existent, and babies with these critical conditions need intervention right after birth. While there are known genetic mutations that lead to these critical heart defects, early perturbations in blood flow can independently lead to critical heart defects. In this paper, we start by comparing the fetal circulation with the neonatal and adult circulation, and reviewing how altered fetal blood flow can be used as a diagnostic tool to plan interventions. We then look at known factors that lead to tetralogy of Fallot and persistent truncus arteriosus: namely early perturbations in blood flow and mutations within VEGF-related pathways. The interplay between physical and genetic factors means that any one alteration can cause significant disruptions during development and underscore our need to better understand the effects of both blood flow and flow-responsive genes.

The Impact of Congenital Heart Disease on Cognitive and Behavioral Functioning

2010 ◽  
Author(s):  
Jo Wray
Keyword(s):  
Congenital Heart Disease ◽  
Blood Flow ◽  
Heart Disease ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Congenital Abnormalities ◽  
Heart Defects ◽  
Survival Rates ◽  
Arterial Circulation ◽  
Great Vessels

Congenital heart disease (CHD) has been defined as “. . . a gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance” (Mitchell, Korones, and Berendes 1971). Congenital heart disease is the most common single group of congenital abnormalities, accounting for about 30% of the total. The incidence is reported as varying between 0.3% and 1% of all live births. Ten to 15% of children with congenital heart defects have more than one cardiac abnormality; up to one-third also have one or more associated noncardiac congenital abnormalities (Wernovsky 2006). Although some forms of CHD are minor and do not require any medical or surgical intervention, others are very complex and may necessitate a series of staged surgical procedures and/or require life-long medications. Significant improvements in medical and surgical techniques have resulted in increasing numbers of children and adults living with CHD, and it is currently anticipated that 80%–85% of children born with CHD today will survive into adulthood (British Cardiac Society 2002). However, although survival rates have improved dramatically over the last 40 years or so, morbidity remains a concern. Congenital heart defects can be broadly subdivided into two groups, based on changes in the circulation. Acyanotic defects may be due to either a left-to-right shunt or to an obstructive lesion; there is no mixing of desaturated blood in the systemic arterial circulation. With cyanotic defects, there may be either increased or diminished pulmonary flow, and desaturated blood enters the systemic arterial circulation, regardless of whether cyanosis is clinically evident. Unsaturated venous blood bypassing the lungs can result in secondary polycythemia, which is a compensatory mechanism to carry more oxygen to the tissues. This causes increased viscosity, which in turn results in sluggish blood circulation and impeded blood flow, particularly in the capillaries. Poor peripheral blood flow and clubbing of the fingers and toes can result, breathlessness and fatigue often result in a reduced exercise tolerance, and growth may be affected.

Cerebrovascular Blood Flow Dynamic Changes in Fetuses with Congenital Heart Disease

2009 ◽  
Vol 25 (1) ◽  
pp. 167-172 ◽  
Author(s):  
Lv Guorong ◽  
Li Shaohui ◽  
Jin Peng ◽  
Lin Huitong ◽  
Li Boyi ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Blood Flow ◽  
Heart Disease ◽  
Congenital Heart ◽  
Flow Dynamic ◽  
Dynamic Changes ◽  
Blood Flow Dynamic

scholarly journals Personalized Genetic Diagnosis of Congenital Heart Defects in Newborns

2021 ◽  
Vol 11 (6) ◽  
pp. 562
Author(s):  
Olga María Diz ◽  
Rocio Toro ◽  
Sergi Cesar ◽  
Olga Gomez ◽  
Georgia Sarquella-Brugada ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart Defects ◽  
Congenital Malformations ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Heart Defects ◽  
Genetic Diagnosis ◽  
Single Nucleotide Variants ◽  
Personalized Approach

Congenital heart disease is a group of pathologies characterized by structural malformations of the heart or great vessels. These alterations occur during the embryonic period and are the most frequently observed severe congenital malformations, the main cause of neonatal mortality due to malformation, and the second most frequent congenital malformations overall after malformations of the central nervous system. The severity of different types of congenital heart disease varies depending on the combination of associated anatomical defects. The causes of these malformations are usually considered multifactorial, but genetic variants play a key role. Currently, use of high-throughput genetic technologies allows identification of pathogenic aneuploidies, deletions/duplications of large segments, as well as rare single nucleotide variants. The high incidence of congenital heart disease as well as the associated complications makes it necessary to establish a diagnosis as early as possible to adopt the most appropriate measures in a personalized approach. In this review, we provide an exhaustive update of the genetic bases of the most frequent congenital heart diseases as well as other syndromes associated with congenital heart defects, and how genetic data can be translated to clinical practice in a personalized approach.

scholarly journals Down syndrome and congenital heart disease: perioperative planning and management

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Dennis R. Delany ◽  
Stephanie S. Gaydos ◽  
Deborah A. Romeo ◽  
Heather T. Henderson ◽  
Kristi L. Fogg ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Cardiac Surgery ◽  
Heart Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Heart Defects ◽  
Careful Consideration ◽  
Children With Down Syndrome ◽  
Perioperative Planning

AbstractApproximately 50% of newborns with Down syndrome have congenital heart disease. Non-cardiac comorbidities may also be present. Many of the principles and strategies of perioperative evaluation and management for patients with congenital heart disease apply to those with Down syndrome. Nevertheless, careful planning for cardiac surgery is required, evaluating for both cardiac and noncardiac disease, with careful consideration of the risk for pulmonary hypertension. In this manuscript, for children with Down syndrome and hemodynamically significant congenital heart disease, we will summarize the epidemiology of heart defects that warrant intervention. We will review perioperative planning for this unique population, including anesthetic considerations, common postoperative issues, nutritional strategies, and discharge planning. Special considerations for single ventricle palliation and heart transplantation evaluation will also be discussed. Overall, the risk of mortality with cardiac surgery in pediatric patients with Down syndrome is no more than the general population, except for those with functional single ventricle heart defects. Underlying comorbidities may contribute to postoperative complications and increased length of stay. A strong understanding of cardiac and non-cardiac considerations in children with Down syndrome will help clinicians optimize perioperative care and long-term outcomes.

scholarly journals Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service

2014 ◽  
Vol 32 (2) ◽  
pp. 159-163 ◽  
Author(s):  
Felipe Alves Mourato ◽  
Lúcia Roberta R. Villachan ◽  
Sandra da Silva Mattos
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Diseases ◽  
Heart Defects ◽  
Descriptive Analysis ◽  
Atrioventricular Septal Defect

OBJECTIVE:To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis.METHODS:Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7.RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1%) and 112 (81.2%) were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age.CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension.

Dilatable Pulmonary Artery Banding Palliation in Congenital Heart Disease

2021 ◽  
Vol 12 (2) ◽  
pp. 213-219
Author(s):  
R. Allen Ligon ◽  
Larry A. Latson ◽  
Mark M. Ruzmetov ◽  
Kak-Chen Chan ◽  
Immanuel I. Turner ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Blood Flow ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Pulmonary Blood Flow ◽  
Congenital Heart ◽  
Balloon Dilation ◽  
Retrospective Chart Review ◽  
Pulmonary Artery Banding ◽  
Surgical Removal

Background: Surgical pulmonary artery banding (PAB) has been limited in practice because of later requirement for surgical removal or adjustment. The aim of this study is to describe our experience creating a dilatable PAB via transcatheter balloon dilation (TCBD) in congenital heart disease (CHD) patients. Methods: Retrospective chart review of adjustable PAB—outline anatomical variants palliated and patient outcomes. Results: Sixteen patients underwent dilatable PAB—median age 52 days (range 4-215) and weight 3.12 kg (1.65-5.8). Seven (44%) of the patients were premature, 11 (69%) had ventricular septal defect(s) with pulmonary over-circulation, four (25%) atrioventricular septal defects, and four (25%) single ventricle physiology. Subsequent to the index procedure: five patients have undergone intracardiac complete repair, six patients remain well palliated with no additional intervention, and four single ventricles await their next palliation. One patient died from necrotizing enterocolitis (unrelated to PAB) and one patient required a pericardiocentesis postoperatively. Five patients underwent TCBD of the PAB without complication—Two had one TCBD, two had two TCBD, and another had three TCBD. The median change in saturation was 14% (complete range 6-22) and PAB diameter 1.7 mm (complete range 1.1-5.2). Median time from PAB to most recent outpatient follow-up was 868 days (interquartile range 190-1,079). Conclusions: Our institution has standardized a PAB technique that allows for transcatheter incremental increases in pulmonary blood flow over time. This methodology has proven safe and effective enough to supplant other institutional techniques of limiting pulmonary blood flow in most patients—allowing for interval growth or even serving as the definitive palliation.

Effect of Variation in Arterial Carbon Dioxide Levels on Cerebral-Somatic Oxygenation in Children with Cyanotic Congenital Heart Disease

2018 ◽  
pp. 1-6
Keyword(s):  
Carbon Dioxide ◽  
Congenital Heart Disease ◽  
Blood Flow ◽  
Heart Disease ◽  
Infrared Spectroscopy ◽  
Congenital Heart ◽  
Near Infrared ◽  
Cyanotic Congenital Heart Disease ◽  
Anesthesia Induction ◽  
Arterial Blood

Background: Hypocapnia is suggested in decreasing pulmonary vascular resistance in cyanotic congenital heart disease patients undergoing definitive repair. But its effects on cerebral and renal circulation are unclear. Hence the effect of changes in arterial blood carbon dioxide tensions (PaCo2 ) on cerebral (ScO2 %) and renal (SsO2 %) oxygenation indices using Near Infrared spectroscopy (NIRS) is examined. Methods: We did a prospective observational study in sixty-eight children who underwent elective cardiac surgery for various cyanotic congenital heart diseases. PaCo2 , ScO2 % and SsO2 % were obtained before induction of anesthesia, after anesthesia induction at normocapnic or mild hypercapnic ventilation (EtCo2 =40 mmHg) and again at hypocapnic ventilation (EtCo2 =30 mmHg). Regression analysis was done between PaCo2 and NIRS-C/ScO2 % and PaCo2 and NIRS-R/SsO2 % at both EtCo2 40 and 30 mmHg. Repeated measure analysis performed to evaluate the significance of change in NIRS-C and NIRS-R from pre-anesthesia induction to when EtCo2 was 40 and then 30 mmHg post anesthesia induction. Results: With decrease in EtCo2 , PaCo2 (p=0.0001), NIRS-C (p=0.0001) and NIRS-R (p=0.0001) decreased significantly. At EtCo2 of 40 and 30 mmHg, PaCo2 had significant positive correlation with NIRS-C (R2 =0.77, p=0.0001 and R2 =0.92, p=0.0001 respectively) and had insignificant correlation with NIRS-R (R2 =0.03, p=0.12 and R2 =0.008, p=0.46 respectively). Significant changes in NIRS-C {p=0.0001} and NIRS-R {p=0.0001} occurred from pre-induction to when EtCo2 was 40 and then to 30 mmHg. Conclusion: A decrease in NIRS-C and NIRS-R is probably from decreased cerebral and splanchnic blood flow during hypocapnic ventilation, leading to demand supply mismatch. Hypocapnic ventilation in cyanotic children has potential to cause cerebral hypoxia. Abbreviations: CCHD: Cyanotic Congenital Heart Disease; QP: Pulmonary blood flow; Do2 : Oxygen delivery; SpO2 : peripheral pulse oximetry; NIRS: Near Infrared Spectroscopy; NIRS-C/ScO2 %: Regional Cerebral Oxygen saturation; NIRS-R/SsO2 %: Regional Somatic/renal Oxygen saturation; HCT: Hematocrit; ECG: Electrocardiography; CPB: cardiopulmonary bypass; TOF: Tetralogy of fallot; BDG: Bidirectional Glenn Shunt; BT shunt: Blalock Taussig shunt; DORV: Double outlet right ventricle; FiO2 : Inspired oxygen concentration; ABG: Arterial blood gas; PaO2 : Arterial oxygen partial pressure; PaCo2 : Arterial carbon dioxide partial pressure; HR: Heart rate; MAP: Mean Arterial Pressure; CVP: Central Venous Pressure

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