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Abstract Background Pulmonary regurgitation is the most common complication after the complete repair of tetralogy of Fallot, and severe pulmonary regurgitation after surgery requires pulmonary valve replacement. In this retrospective observational, cross-sectional study, we included a total of 56 children aged 6 years or younger who underwent complete repair of TOF at Shahid Rajaei Cardiovascular Medical and Research Center in Tehran, Iran. Preoperative dual-source computed tomography was used to measure the McGoon ratio and Nakata index. The patients were divided into two groups based on the severity of postoperative pulmonary regurgitation, as estimated by trans-thoracic echocardiography: the severe pulmonary regurgitation group and the non-severe pulmonary regurgitation group. The McGoon ratio and Nakata index were then compared between the two groups. Results When comparing the two groups, we found that the corrected right pulmonary artery diameter, main pulmonary artery diameter, and McGoon ratio in the non-severe pulmonary regurgitation group were higher than in the severe pulmonary regurgitation group. However, none of these differences were statistically significant. Additionally, other variables, including the corrected left pulmonary artery diameter and Nakata index, showed higher measurements in children with severe pulmonary regurgitation, but again, the differences were not statistically significant. Conclusions This study indicates that pulmonary arteries diameter, Nakata index, and McGoon ratio were not significantly correlated with the severity of pulmonary regurgitation after the complete repair of tetralogy of Fallot.

Background: Pulmonary regurgitation is the most common complication after the complete repair of tetralogy of Fallot, and severe pulmonary regurgitation after surgery requires pulmonary valve replacement. In this retrospective observational, cross-sectional study, we included a total of 56 children aged 6 years or younger who underwent complete repair of TOF at Shahid Rajaei Cardiovascular Medical and Research Center in Tehran, Iran. Preoperative dual-source computed tomography was used to measure the McGoon ratio and Nakata index. The patients were divided into two groups based on the severity of postoperative pulmonary regurgitation, as estimated by trans-thoracic echocardiography: the severe pulmonary regurgitation group and the non-severe pulmonary regurgitation group. The McGoon ratio and Nakata index were then compared between the two groups. Results: When comparing the two groups, we found that the corrected right pulmonary artery diameter, main pulmonary artery diameter, and McGoon ratio in the non-severe pulmonary regurgitation group were higher than in the severe pulmonary regurgitation group. However, none of these differences were statistically significant. Additionally, other variables, including the corrected left pulmonary artery diameter and Nakata index, showed higher measurements in children with severe pulmonary regurgitation, but again, the differences were not statistically significant. Conclusions: This study indicates that pulmonary arteries diameter, Nakata index, and McGoon ratio were not significantly correlated with the severity of pulmonary regurgitation after the complete repair of tetralogy of Fallot. [ABSTRACT FROM AUTHOR]

Objective To assess the extent and the characteristics of hospital admissions in registered adult patients with congenital heart disease. Design Observational cohort study. Setting The Netherlands. Patients 5798 adult patients with congenital heart disease from the Dutch CONCOR national registry linked to the Dutch National Medical Registration (Prismant). Main outcome measures All hospital admissions from the years 2001 up until 2006. Results During 28⇔990 patient-years, 2908 patients (50%) were admitted to hospital. Median age at admission was 39 years (range 18-86 years); 46% were male. Admission rate in CONCOR patients was high among all ages (range 11-68%) and exceeded that of the general Dutch population two to three times; this difference was most pronounced in the older age groups. Altogether there were 8916 admissions, 5411 (61%) of which were for cardiovascular indications. Among cardiovascular admissions, referrals for arrhythmias were most common (31%). Of 4926 interventions, 2459 (50%) were cardiovascular, most often reparative interventions or cardioversion (53%). Most non-cardiovascular admissions were obstetric. Among defects, univentricular heart and tricuspid atresia had the highest incidence and duration of admission. Conclusions Healthcare utilisation in registered and medically supervised adult patients with congenital heart disease is high and increases with age. Admission rates are at least two times higher than in the general population, and most marked in the older age groups. With the ageing of this population, a major increase in healthcare utilisation is imminent in the near future. Timely preparation of healthcare resources is crucial to sustain optimal care. [ABSTRACT FROM AUTHOR]

Objective: Patients with tetralogy of Fallot (TOF) have high survival rates 30 years after surgical repair. Many patients experience pregnancy; however, the effects of pregnancy on the long-term cardiovascular outcome are not well known. We investigated the association of pregnancy and cardiac function with occurrence of ventricular arrhythmia (VA) in women with TOF., Methods: We recruited 80 women with repaired TOF from the national database. Holter monitoring or implanted devices detected VA, defined as non-sustained or sustained ventricular tachycardia or aborted cardiac arrest. All patients underwent echocardiography. Blood tests included NT-proBNP (N-terminal pro-brain natriuretic peptide)., Results: 55 (69%) women had experienced pregnancy. Mean age was lower in nulliparous compared with those with children (30±9 vs 40±9, p<0.01).VA had occurred in 17 (21%) women. Prevalence of VA was higher in women who had experienced pregnancy (n=16, 94%) compared with nulliparous (n=1, 6%) (p=0.02), also when adjusted for age (OR 12.9 (95% CI 1.5 to 113.2), p=0.02).Right ventricular mechanical dispersion was more pronounced in patients with VA (50±8 ms vs 39±14 ms, p=0.01, age-adjusted OR 2.1 (95% CI 1.3 to 7.5), p=0.01). NT-proBNP was also a marker of VA (211 ng/L (127 to 836) vs 139 ng/L (30 to 465), p=0.007). NT-proBNP >321 ng/L (normal values <170 ng/L) detected women with VA (p=0.019), also independent of age (OR 7.2 (95% CI 1.7 to 30.1), p=0.007)., Conclusion: Pregnancy was associated with higher prevalence of VA among women with TOF. Right ventricular mechanical dispersion and NT-proBNP were age-independent markers of VA. These may have importance for pregnancy counselling and risk stratification., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: Left ventricular systolic dysfunction (LVSD) in adult survivors of tetralogy of Fallot (TOF) repair in childhood has been observed, although the relationship with long-term outcome remains inadequately described., Methods: A cohort of 44 consecutive adult patients with TOF repair in childhood were followed prospectively from January 2001 through June 2016. LVSD was defined as an echocardiographically derived left ventricular (LV) ejection fraction <0.55. Clinical and demographic characteristics in patients with and without LVSD were compared. Event-free survival (all-cause death or hospitalisation) was estimated using the product limit method., Results: The average time from childhood surgical repair to cohort inception was similar between groups (LVSD, 33.7±12.7 years; normal LV function, 36.1±14.9 years; P=0.62) as were their mean ages (LVSD, 36.5±14.5 years; normal LV function, 40.7±15.2 years; P=0.73). Patients with LVSD (n=13) had more prior surgeries, more frequent history of significant pulmonic regurgitation, right ventricular systolic dysfunction and more implantable cardiac devices. Over a total observation time of 15.5 years, patients with LVSD were at significantly higher risk of all-cause death or hospitalisation (P=0.006). Onset of LVSD frequently preceded an adverse outcome., Conclusions: In this cohort of adult patients with TOF repair in childhood followed for a total of 550 patient-years, the frequency of LVSD was 30%. LVSD was associated with lower event-free survival. The appearance of LVSD many years after TOF repair may herald the onset of an adverse outcome., Competing Interests: Competing interests: None declared.

Objective Right ventricular (RV) dysfunction is common in patients with corrected tetralogy of Fallot (cToF). Abnormalities in the temporal pattern of RV mechanical activation have been observed in patients with cToF, but the relationship with RV performance remains unclear. This study characterises RV performance and the temporal sequence of RV deformation in patients with cToF and healthy controls. Study design 37 patients with cToF were compared with 18 controls. Using two-dimensional speckle tracking analysis, global RV strain was assessed. In addition, time to peak strain and the time difference between RV inlet and RV outlet (RV time delay) was assessed. Main outcome measure The relation between RV performance and RV time delay was assessed with linear regression analysis. Results RV strain was reduced in patients compared with controls (⇔'20.9±4.3% vs ⇔'30.7±3.4%, p<0.001). Time to peak strain at the RV inlet showed no differences between patients with cToF and controls (406±55⇔…ms vs 405±67⇔…ms, p=0.429), whereas time to peak strain at the RV outlet was significantly delayed in patients with cToF (339±75⇔…ms vs 262±85⇔…ms, p=0.003). Consequently, RV time delay was significantly shorter in patients with cToF than in controls (66±48⇔…ms vs 143±53⇔…ms, p<0.001). A close relation between RV time delay and RV strain was observed (r=⇔'0.70, p<0.001). Conclusion In patients with cToF, RV outlet deformation is delayed, causing a reduction in RV time delay which is significantly related to impairment in RV performance. [ABSTRACT FROM AUTHOR]

Background Restrictive right ventricular (RV) physiology is a phenomenon considered potentially beneficial when detected in children and adolescents with repaired tetralogy of Fallot (ToF). It is typically characterised by antegrade flow in the pulmonary artery in late diastole at the time of atrial contraction. However, little is known about the impact of restrictive physiology on intrinsic diastolic RV function or lusitropy. Methods Diastolic function was prospectively assessed at baseline level and during dobutamine infusion using the pressure-volume conductance system. End diastolic forward flow in the pulmonary artery was measured by MRI. Results Twenty-five patients aged 17.9±7.5 years were studied. Although the end diastolic RV pressure, the time constant of isovolumic RV relaxation (↔) and dP/dtmin were similar between both groups, the slope of the end diastolic pressure-volume relationship (Eed) was significantly higher in the restrictive group indicating increased diastolic stiffness in these patients (0.14±0.05 vs 0.06±0.01, p=0.03). Dobutamine stress, however, led to an increase in Eed in the entire ToF cohort indicating an abnormal diastolic response to catecholamines in these patients. Conclusions Diastolic RV stiffness of repaired ToF patients with restrictive physiology is increased. The lusitropic response of the RV to β adrenergic agents is abnormal after ToF repair regardless of whether restrictive physiology is present or not. This has potential implications, particularly for postoperative drug management. [ABSTRACT FROM AUTHOR]

Heart failure (HF) is a growing public health concern as a consequence of the ageing of the population and the improved survival of patients with HF. HF is defined as impaired organ perfusion and/or high filling pressure. It is a systemic and chronic disease and as such involves many organs, not least the liver and kidney. The complex vascular system of the liver and its high metabolic activity render it vulnerable to circulation disturbances and trigger many molecular and haemodynamic changes in patients. There are many studies describing the impact of liver disease on patient outcomes. Hepatic dysfunction is commonly seen in HF patients and is closely correlated with a poor outcome. Knowledge about the mechanisms and impacts of liver disease in HF helps us to know the stage of the disease and treat it properly. Moreover, many drugs and toxins that are metabolised in the liver and contribute to drug interactions should also be taken into account when prescribing medication for HF patients. In light of the above-mentioned points, the authors have compiled this review on congestive hepatopathy with the aim of providing physicians and cardiologists with a succinct and useful guide on the role of the liver in HF.