Abstract Objectives To evaluate the effects of endothelin inhibitors (ERAs) on hemodynamic and functional parameters in patients post-Fontan procedure with high pulmonary vascular resistance (PVR). ...Methods Among our cohort of patients with Fontan circulation, 8 children, 8 adolescents, and 8 adults had PVR ≥2 WU*m2 . These patients were treated with ERAs (minors with bosentan, adults with macitentan) and reevaluated after 6 months. Pre- and posttreatment hemodynamic variables were assessed by cardiac catheterization. Functional capacity was evaluated by cardiopulmonary exercise testing (CPET). Our primary endpoint was to obtain a reduction of PVR; the secondary endpoint was to obtain an improvement of functional capacity. Results Under treatment, New York Heart Association class improved for adolescents and adults. PVR decreased ( P = .01) in all groups: in children from the median value 2.3 (interquartile range 2.0-3.1) to 1.9 (1.4-2.3) WU*m2 , in adolescents from 2.3 (2.1-2.4) to 1.7 (1.4-1.8) WU*m2 , and in adults from 2.8 (2.0-4.7) to 2.1 (1.8-2.8)WU*m2 . In 71% of patients, PVR fell to less than 2 WU*m2 . Cardiac index increased in adolescents from 2.6 (2.4-3.3) to 3.6 (3.4-4.3) L/min/m2 , P = .04, and in adults from 2.1 (2.0-2.3) to 2.8 (2.3-4.7) L/min/m2 , P = .03. CPET showed that only adolescents displayed a significant functional improvement. Anaerobic threshold improved from 17 (13-19) to 18 (13-20) mL/kg/min, P = .03; oxygen consumption and VO2 max increased from 1.3 (1.0-1.6) to 1.7 (1.1-1.9) L/min, P = .02 and from 25 (21-28) to 28 (26-31) L/min, P = .02, respectively. Oxygen pulse increased from 7.9 (5.7-10.4) to 11.2 (8.2-13.0) L/beat, P = .01. Conclusions This is the first study that assesses by cardiac catheterization and CPET the effects of ERA in patients with Fontan circulation with increased PVR. These results suggest that ERAs might provide most pronounced hemodynamic and functional improvement in adults and adolescents.
Abstract
OBJECTIVES
Italy has been one of the countries most severely affected by the coronavirus disease 2019 (COVID-19). The Italian government was forced to introduce quarantine measures quickly, ...and all elective health services were stopped or postponed. This emergency has dramatically changed the management of paediatric and adult patients with congenital heart disease. We analysed data from 14 Italian congenital cardiac surgery centres during lockdown, focusing on the impact of the pandemic on surgical activity, patients and healthcare providers and resource allocation.
METHODS
Fourteen centres participated in this study. The period analysed was from 9 March to 4 May. We collected data on the involvement of the hospitals in the treatment of patients with COVID-19 and on limitations on regular activity and on the contagion among patients and healthcare providers.
RESULTS
Four hospitals (29%) remained COVID-19 free, whereas 10 had a 39% reduction in the number of beds for surgical patients, especially in the northern area. Two hundred sixty-three surgical procedures were performed: 20% elective, 62% urgent, 10% emergency and 3% life-saving. Hospital mortality was 0.4%. Compared to 2019, the reduction in surgical activity was 52%. No patients operated on had positive test results before surgery for severe acute respiratory syndrome coronavirus 2, the virus responsible for COVID-19. Three patients were infected during the postoperative period. Twenty-nine nurses and 12 doctors were infected. Overall, 80% of our infected healthcare providers were in northern centres.
CONCLUSIONS
Our study shows that the pandemic had a different impact on the various Italian congenital cardiac surgery centres based on the different patterns of spread of the virus across the country. During the lockdown, the system was able to satisfy all emergency clinical needs with excellent results.
Starting from Wuhan (China) in December 2019 1, the disease called coronavirus disease 2019 (COVID-19) has rapidly become a worldwide pandemic with currently over 12 million cases globally (15 June).
Abstract Background In patients with Fontan circulation, the liver is profoundly affected by chronic venous stasis. Little is known about early hepatic changes in this population. Methods We ...performed echocardiography, abdominal ultrasound, liver elastography, cardiac catheterization, esophago-gastro-duodenoscopy and calculated MELD-XI score in 64 Fontan patients (69% minors), at an interval of 1–15 years since Fontan. Results Cardiac output remained stable in the first 5 years after Fontan, then significantly decreased (r = − 0.45, p(r = 0) = 0.003). NYHA class significantly increased after Fontan. Patients in NYHA class II/III (n = 21, 14 minors) had significantly higher hepatic pressures, but normal ventricular function and pulmonary vascular resistances (PVR). Patients with pulmonary arterial pressure (PAP) ≥ 15 mm Hg (n = 12, 6 minors) and those with PVR ≥ 2 WU*m2 (n = 27, 25 minors), had higher hepatic pressures (p < 0.0001), a higher incidence of liver collaterals and/or esophageal varices (p < 0.0001) and splenomegaly (p < 0.02). Liver stiffness (LS) was elevated in most patients (median, 25th–75th percentile:17.3 KPa, 14.1–21.4). It rapidly increased during the first 5-years after Fontan, compared to the following 5-years (from 12.2 KPa, 9.8–14.1 to 17.5 KPa, 14.3–24.5, p = 0.007), then remained stable (19.1 KPa, 16.9–22.6, p = 0.60). MELD-XI score increased linearly with the time interval since Fontan (r = 0.31, p(r = 0) = 0.01). For patients above 12 years we found a linear correlation between LS and MELD-XI score in the 6–15 years period after Fontan (r = 0.40. p(r = 0) = 0.04). The overall incidence of established liver cirrhosis was 22%. Conclusions This is the largest study showing that Fontan circulation prompts early, progressive and eventually irreversible liver damage. Precautions should be taken immediately after Fontan, to protect this fragile population.
Background Previous studies have shown that older Fontan patients and those with an underlying morphologically right ventricle have lower exercise capacity. We sought to assess the natural history of ...exercise capacity after the Fontan operation in individual patients, and to identify the factors influencing the rate of decrease of exercise capacity over time. Methods We studied, longitudinally, 53 Fontan patients with cardiopulmonary exercise tests (average 3.2 ± 1.1 tests for each patient). Age at the first test was 14 ± 6 years. Time intervals between tests ranged from 1 to 16 years (average, 7.7 ± 4.0 years). The morphology of the functionally single ventricle was left in 29 patients (55%) and right in 24 patients (45%). Twenty-two patients had undergone a total cavopulmonary connection (TCPC) at a mean age of 5.9 ± 2.8 years. Thirty-one patients had undergone an atriopulmonary or an atrioventricular connection at an age of 6.9 ± 4.4 years. Exercise capacity was expressed as percentage of predicted peak oxygen uptake (V o2 ). Results Overall, peak V o2 decreased at a rate of −2.6 ± 2.7%/year. Ventricular morphology (r = 0.525, p = 0.0001) and type of Fontan operation (r = 0.381, p = 0.0057) appeared as the only predictors of the rate of decrease of peak V o2 at multivariate analysis. Patients with an underlying left ventricular morphology (−1.7 ± 2.0 vs −3.7 ± 3.2%/year, p = 0.005), and those who underwent a TCPC (−1.9 ± 1.8 vs −3.3 ± 3.2%/year, p = 0.042), had the lowest rate of decrease in peak V o2. Conclusions Exercise capacity progressively declines in Fontan subjects. The decline of exercise capacity seems to be slower in patients with an underlying left ventricular morphology and in those who received a TCPC.
Objective: To analyse the long-term patency of coronary arteries after neonatal arterial switch operation (ASO). Methods: A retrospective study of the operative reports, follow-up and postoperative ...catheterisation data of 119 patients, who underwent the great arteries (TGA) repair since 1991, has been carried out. Patient population: Among the 133 survivors of the 137 ASOs performed between 1991 and 2007, 119 patients have been studied by routine control cardiac catheterisation and form the study population. Median time between repair and the coronary angiography was 2.9 ± 1.9 years. A comparison between the eight patients (6.7% out of the entire study population), known to have postoperative coronary obstructions (group I) and the rest of the cohort with angiographic normal coronary vessels (group II) was performed by univariate analysis of variance and logistic regression models. One patient had surgical plasty of the left coronary main stem with subsequent percutaneous angioplasty, three patients had primary coronary stent implantation and four patients had no further intervention at all. In group I, all but one patient denied symptoms of chest pain and echocardiography failed to show any difference between the two groups in terms of left ventricular systolic function (ejection fraction group I 61 ± 2% vs 62 ± 6% of group II, p = 1.0). Results: The association of coronary obstruction with complex native coronary anatomy (Yacoub type B to E) was evident at both univariate (62% of group I vs 22% of group II, p = 0.04) and logistic regression (p = 0.007, odds ratio (OR) 8.1) models. The type of coronary reimplantation (i.e., coronary buttons on punch vs trap-door techniques) was similar between the two groups (punch reimplantation in 25% of patients of group I vs 31% of group II, p = 0.1) as was the relative position of the great vessels (aorta anterior in 100% of patients of group I vs 96% of group II; univariate, p = 0.1). Conclusions: The late outcome in terms of survival and functional status after ASO is excellent. Nevertheless, the risk of a clinically silent late coronary artery obstruction of the reimplanted coronary arteries warrants a prolonged follow-up protocol involving invasive angiographic assessment.
Capillary leak syndrome is a critical condition occasionally occurring posttransplant and is characterized by acute endothelial hyperpermeability leading to systemic protein-rich fluid extravasation ...and consequent hypovolemia, hypoperfusion, and acute kidney injury. Treatment is merely supportive and is based on osmotic drugs, diuretics, continuous renal replacement therapy, and surgical drainage. However, removal of the underlying inflammatory cause is mandatory to achieve stable resolution. Herein, we report the first successful treatment with colchicine in 2 life-threatening pediatric cases of capillary leak syndrome with renal failure occurring after transplant (heart and bone marrow) and unresponsive to any other line of therapy. Both cases were only palliated by supportive therapy and revealed an impressively rapid response to colchicine both in terms of diuresis and clinical condition recovery, allowing for the cessation of renal replacement therapy in a few hours. In both patients, colchicine was temporarily discontinued for transient leukopenia (attributed to an additive effect with mycophenolate mofetil), resulting in extravasation, and renal failure recurrence was restored only after colchicine reintroduction. Although the association of colchicine with an immunosuppressive drug was formerly contraindicated, no other adverse events were noted when using a minimized dose. Both patients are now maintaining a good renal function without recurrence of extravasation after 6 months of follow-up. In conclusion, this strikingly positive experience forces physicians to consider this old and cost-effective drug as a new, powerful rescue tool in such critical cases.
Background and Aim of the Study
Outcomes after repair of tetralogy of Fallot (TOF) are good with either a transventricular (TV) or transatrial (TA) approach. We sought to determine if there is a ...relationship between the TV or TA approach and right ventricular (RV) function, and the role of residual pulmonary regurgitation (PR) on the long‐term outcomes.
Methods
This was a retrospective cohort multicentric study on survivors after surgical repair of TOF (TA versus TV approach, ±transannular patch) between 1990 and 2004. All patients underwent magnetic resonance imaging to assess RV volume, function, and PR. Patients were matched for length of follow‐up and age. Clinical adverse events were retrieved from institutional databases.
Results
Seventy‐nine patients (TA/TV = 37/42, median age 0.3 and 1.0 yrs, respectively) were included. At a median follow‐up of 16.6 years (12.5‐20.3), there were no differences in freedom from reintervention (either catheter or surgical), RV volumes, function, and PR between the TA and TV groups. Pulmonary valve (PV) replacement was significantly less frequent in the TA subgroup (P = 0.033) and patients with a preserved PV showed significantly lower RV volumes and less adverse events at follow‐up.
Conclusions
There is no significant difference in RV volumes and function between the TA and TV. However, the TA approach seems to be protective against PV replacement in the long‐term. When PV is not preserved at repair, residual pulmonary regurgitation is a significant cause of late RV dysfunction and dilation, and is associated with a higher rate of late adverse events.
Children undergoing Ross operation were expected to have longer autograft, but shorter homograft durability compared with adults. In order to define the outcome in the second decade after Ross ...operation in children, a nationwide review of 23 years of experience was undertaken.
305 children underwent Ross operation in 11 paediatric units between 1990 and 2012. Age at surgery was 9.4±5.7 years, indication aortic stenosis in 103 patients, regurgitation in 109 and mixed lesion in 93. 116 (38%) patients had prior procedures. Root replacement was performed in 201 patients, inclusion cylinder in 14, subcoronary grafting in 17 and Ross-Konno in 73.
There were 10 (3.3%) hospital and 12 late deaths (median follow-up 8.7 years). Survival was 93±2% and 89±3% and freedom from any reoperation was 76±3% and 67±6% at 10 and 15 years. 34 children had autograft 37 reoperations (25 replacement, 12 repair): three required transplantation after reoperation. Freedom from autograft reoperation was 86±3% and 75±6% at 10 and 15 years. 32 children had right heart redo procedures, and only 25 (78%) conduit replacements (15-year freedom from replacement, 89±4%). Prior operation (p=0.031), subcoronary implant (p=0.025) and concomitant surgical procedure (p=0.004) were risk factors for left heart reoperation, while infant age (p=0.015) was for right heart. The majority (87%) of late survivors were in NYHA class I, 68% free from medication and six women had pregnancies.
Despite low hospital risk and satisfactory late survival, paediatric Ross operation bears substantial valve-related morbidity in the first two decades. Contrary to expectation, autograft reoperation is more common than homograft.
Abstract
OBJECTIVES
Repair of Tetralogy of Fallot (TOF) has currently excellent results with either transventricular or transatrial approach. However, it is unclear as to which has better late ...outcomes and what role of residual pulmonary valve (PV) regurgitation in the long term is. We report on late clinical outcomes after repair in a large series of patients with TOF, focusing on the type of surgical technique.
METHODS
This analysis is a retrospective multicentre study on patients undergoing TOF repair in infancy. The exclusion criteria of the study were TOF with pulmonary atresia or absent PV.
RESULTS
We selected 720 patients who had undergone TOF repair (median age 5.7 months, interquartile range 3.7–11.7). Preoperative cyanotic spells occurred in 18%. A transatrial repair was performed in 433 (60.1%) patients. The PV was preserved in 249 (35%) patients, while the right ventricular outflow tract was reconstructed with a transannular patch (60.4%) or a conduit (4.6%) in the rest of the patients. At a median follow-up of 4 years (range 1–21, 86% complete), 10 (1.6%) patients died, while 39 (6.3%) patients required surgical reoperation and 72 (11.7%) patients required an interventional procedure. The propensity match analysis showed that the incidence of postoperative complications and adverse events at follow-up were significantly increased in patients undergoing transventricular approach repair with transannular patch (P = 0.006) and PV preservation was a significant protective factor against postoperative complications (P = 0.009, odds ratio 0.5) and late adverse events (P = 0.022).
CONCLUSIONS
Surgical repair of TOF in infancy is a safe procedure, with good late clinical outcomes. However, transatrial approach and PV preservation at repair are associated with lower early and late morbidity.
The surgical treatment of neonatal aortic coarctation is usually accomplished with a termino-terminal anastomosis or a subclavian flap. The use of a patch to enlarge the isthmal narrowing may be an ...alternative but is frequently complicated by aneurysmal dilatation on the aortic wall opposite to the patch, probably because it disrupts the vascular anatomic integrity. Extracellular matrix patches promise to restore the original tissue structure and could therefore be a valid alternative to other materials. We describe an aortic coarctation with an uncommon anatomic aspect treated with a CorMatrix (CorMatrix, Alpharetta, GA) extracellular matrix patch.
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