Impaired Pulmonary Function is an Additional Potential Mechanism for the Reduction of Functional Capacity in Clinically Stable Fontan Patients.

Central factors negatively affect the functional capacity of Fontan patients (FP), but "non-cardiac" factors, such as pulmonary function, may contribute to their exercise intolerance. We studied the pulmonary function in asymptomatic FP and its correlations with their functional capacity. Pulmonary function and cardiopulmonary exercise tests were performed in a prospective study of 27 FP and 27 healthy controls (HC). Cardiovascular magnetic resonance was used to evaluate the Fontan circulation. The mean age at tests, the mean age at surgery, and the median follow-up time of FP were 20(±6), 8(±3), and 11(8-17) years, respectively. Dominant ventricle ejection fraction was within normal range. The mean of peak VO2 expressed in absolute values (L/min), the relative values to body weight (mL/kg/min), and their predicted values were lower in FP compared with HC: 1.69 (±0.56) vs 2.81 (±0.77) L/min; 29.9 (±6.1) vs 41.5 (±9.3) mL/kg/min p < 0.001 and predicted VO2 Peak [71% (±14) vs 100% (±20) p < 0.001]. The absolute and predicted values of the forced vital capacity (FVC), forced expiratory volume in one second (FEV1), inspiratory capacity (IC), total lung capacity (TLC), diffusion capacity of carbon monoxide of the lung (DLCO), maximum inspiratory pressure (MIP), and sniff nasal inspiratory pressure (SNIP) were also significantly lower in the Fontan population compared to HC. An increased risk of restrictive ventilatory pattern was found in patients with postural deviations (OD:10.0, IC:1.02-97.5, p = 0.042). There was a strong correlation between pulmonary function and absolute peak VO2 [FVC (r = 0.86, p < 0.001); FEV1 (r = 0.83, p < 0.001); IC (r = 0.84, p < 0.001); TLC (r = 0.79, p < 0.001); and DLCO (r = 0.72, p < 0.001). The strength of the inspiratory muscles in absolute and predicted values was also reduced in FP [-79(±28) vs -109(±44) cmH2O (p = 0.004) and 67(±26) vs 89(±36) % (p = 0.016)]. Thus, we concluded that the pulmonary function was impaired in clinically stable Fontan patients and the static and dynamic lung volumes were significantly reduced compared with HC. We also demonstrated a strong correlation between absolute Peak VO2 with the FVC, FEV1, TLC, and DLCO measured by complete pulmonary test.

Functional Performance of Different Venous Limb Options in Simulated Neonatal/Pediatric Cardiopulmonary Bypass Circuits.

OBJECTIVE: Hemodilution is a concern in cardiopulmonary bypass (CPB). Using a smaller dual tubing rather than a single larger inner diameter (ID) tubing in the venous limb to decrease prime volume has been a standard practice. The purpose of this study is to evaluate these tubing options. METHODS: Four different CPB circuits primed with blood (hematocrit 30%) were investigated. Two setups were used with two circuits for each one. In Setup I, a neonatal oxygenator was connected to dual 3/16" ID venous limbs (Circuit A) or to a single 1/4" ID venous limb (Circuit B); and in Setup II, a pediatric oxygenator was connected to dual 1/4" ID venous limbs (Circuit C) or a single 3/8" ID venous limb (Circuit D). Trials were conducted at arterial flow rates of 500 ml/min up to 1500 ml/min (Setup I) and up to 3000 ml/min (Setup II), at 36°C and 28°C. RESULTS: Circuit B exhibited a higher venous flow rate than Circuit A, and Circuit D exhibited a higher venous flow rate than Circuit C, at both temperatures. Flow resistance was significantly higher in Circuits A and C than in Circuits B (P<0.001) and D (P<0.001), respectively. CONCLUSION: A single 1/4" venous limb is better than dual 3/16" venous limbs at all flow rates, up to 1500 ml/min. Moreover, a single 3/8" venous limb is better than dual 1/4" venous limbs, up to 3000 ml/min. Our findings strongly suggest a revision of perfusion practice to include single venous limb circuits for CPB.

Lessons Learned From a Critical Analysis of the Fontan Operation Over Three Decades in a Single Institution.

BACKGROUND: The Fontan operation (FO) has evolved and many centers have demonstrated improved results relative to those from earlier eras. We report a single-institution experience over three decades, describing the outcomes and evaluating risk factors. METHODS: Successive patients undergoing primary FO were divided into era I (1984-1994), era II (1995-2004), and era III (2005-2014). Clinical and operative notes were reviewed for demographic, anatomic, and procedure details. End points included early and late mortality and a composite of death, heart transplantation (HTX), or Fontan takedown. RESULTS: A total of 420 patients underwent 18 atriopulmonary connections, 82 lateral tunnels (LT), and 320 extracardiac conduit (EC) Fontan procedures. Forty-six (11%) patients died; early and late mortality were 7.9% and 3.1%, respectively. Eight (1.9%) patients underwent HTX, 11 (2.6%) underwent Fontan conversion to EC, and 1 (0.2%) takedown of EC to bidirectional Glenn shunt. Prevalence of concomitant valve surgery ( P < .001) and pulmonary artery reconstruction ( P < .001) differed over the eras. Preoperative valve regurgitation was associated with likelihood of early mortality (odds ratio [OR] = 3.5, P = .002). Embolic events (OR = 1.9, P = .047), preoperative valve regurgitation (OR = 2.3, P = .029), diagnosis of unbalanced atrioventricular canal defect (OR = 1.14, P = .03), and concomitant valve replacement (OR = 6.9, P = .001) during the FO were associated with increased risk of the composite end point (death, HTX, or takedown). CONCLUSION: Technical modifications did not result in improved results across eras, due in part to more liberal indications for surgery in the recent years. Valve regurgitation, unbalanced atrioventricular canal, embolic events, or concomitant valve replacement were associated with FO failure.

Risk factors associated with mortality and interventions in 472 neonates with interrupted aortic arch: a Congenital Heart Surgeons Society study.

OBJECTIVE: We sought to determine the prevalence of outcomes and associated patient and management factors for neonates with interrupted aortic arch. METHODS: From 1987 to 1997, a total of 472 neonates were enrolled prospectively from 33 institutions. Competing risks methodology was used to determine simultaneous risk and associated incremental risk factors for death, initial and subsequent left ventricular outflow tract procedures, and arch reinterventions. RESULTS: Overall survival was 59% at 16 years after study entry but improved with successive birth cohort. In general, risk factors for death in each of the competing risks analyses included lower birth weight, younger age at study entry, type B interrupted aortic arch, and major associated cardiac anomalies. Of 453 patients who had interrupted aortic arch repair, after 16 years 33% had died and 28% had undergone an arch reintervention. Reintervention was more likely for those who had truncus arteriosus repair, interrupted aortic arch repair by a method other than direct anastomosis with patch augmentation, and the use of polytetrafluoroethylene as either an interposition graft or a patch. From study entry, competing risks after 16 years showed that 28% had died and 34% had undergone an initial left ventricular outflow tract procedure. Initial left ventricular outflow tract procedure was more likely for those with single ventricle, type B interrupted aortic arch, bicuspid aortic valve, or anomalous right subclavian artery. Among those who had undergone an initial left ventricular outflow tract procedure, after 16 years 37% had died and 28% had undergone a second procedure. CONCLUSION: Anatomic features affect mortality and initial left ventricular outflow tract procedures, whereas characteristics of the arch repair affect arch reintervention.

Barriers to development: pushing the boundaries.

Why some countries have failed to create democracy, wealth and happiness for their people is one of the great questions of our time. This essay encompasses a description of the multiple barriers to development that may have different characteristics, according to the context and the social structures that maintain these conditions. It would be arrogant to pretend to have all the solutions for these problems.

ECMO: Improving our Results by Chasing the Rabbits.

As Marcelo Giugale published in the Financial Times, Latin America, on the whole, has not excelled at innovation - doing the same things in a new and better way or at doing new things. It has been slow to acquire, adopt and adapt technologies by this time available in other places[1]. Although extracorporeal membrane oxygenation (ECMO) is not a new technology, its use in Latin America is not widespread as needed. Furthermore, we still have a number centers doing ECMO, not reporting their cases, lacking a structured training program and not registered with the extracorporeal life support organization (ELSO). With this scenario, and accepting that ECMO is the first step in any circulatory support program, it is difficult to anticipate the incorporation of new and more complex devices as the technologically advanced world is currently doing. However, the good news is that with the support of experts from USA, Europe and Canada the results in Latin America ELSO'S centers are improving by following its guidelines for training, and using a standard educational process. There is no doubt that we can learn a great deal from the high velocity organizations - the rabbits - whom everyone chases but never catches, that manage to stay ahead because of their endurance, responsiveness, and their velocity in self-correction[2].

Cardiac surgery: the infinite quest. Part III--pediatric cardiac surgery: a discipline on its own.

Although there are common grounds with adult cardiac surgery, it is important to understand the differences in the business plan, paths, manpower, mindset, training, and infrastructure that are essential in those institutions where pediatric cardiac surgery can and should be performed. Time to start thinking, it is not what we can do, but should we do it?

Functional performance of different venous limb options in simulated neonatal/pediatric cardiopulmonary bypass circuits

Abstract Objective: Hemodilution is a concern in cardiopulmonary bypass (CPB). Using a smaller dual tubing rather than a single larger inner diameter (ID) tubing in the venous limb to decrease prime volume has been a standard practice. The purpose of this study is to evaluate these tubing options. Methods: Four different CPB circuits primed with blood (hematocrit 30%) were investigated. Two setups were used with two circuits for each one. In Setup I, a neonatal oxygenator was connected to dual 3/16" ID venous limbs (Circuit A) or to a single 1/4" ID venous limb (Circuit B); and in Setup II, a pediatric oxygenator was connected to dual 1/4" ID venous limbs (Circuit C) or a single 3/8" ID venous limb (Circuit D). Trials were conducted at arterial flow rates of 500 ml/min up to 1500 ml/min (Setup I) and up to 3000 ml/min (Setup II), at 36°C and 28°C. Results: Circuit B exhibited a higher venous flow rate than Circuit A, and Circuit D exhibited a higher venous flow rate than Circuit C, at both temperatures. Flow resistance was significantly higher in Circuits A and C than in Circuits B (P<0.001) and D (P<0.001), respectively. Conclusion: A single 1/4" venous limb is better than dual 3/16" venous limbs at all flow rates, up to 1500 ml/min. Moreover, a single 3/8" venous limb is better than dual 1/4" venous limbs, up to 3000 ml/min. Our findings strongly suggest a revision of perfusion practice to include single venous limb circuits for CPB.

ECMO: Improving our Results by Chasing the Rabbits

ABSTRACT As Marcelo Giugale published in the Financial Times, Latin America, on the whole, has not excelled at innovation - doing the same things in a new and better way or at doing new things. It has been slow to acquire, adopt and adapt technologies by this time available in other places[1]. Although extracorporeal membrane oxygenation (ECMO) is not a new technology, its use in Latin America is not widespread as needed. Furthermore, we still have a number centers doing ECMO, not reporting their cases, lacking a structured training program and not registered with the extracorporeal life support organization (ELSO). With this scenario, and accepting that ECMO is the first step in any circulatory support program, it is difficult to anticipate the incorporation of new and more complex devices as the technologically advanced world is currently doing. However, the good news is that with the support of experts from USA, Europe and Canada the results in Latin America ELSO'S centers are improving by following its guidelines for training, and using a standard educational process. There is no doubt that we can learn a great deal from the high velocity organizations - the rabbits - whom everyone chases but never catches, that manage to stay ahead because of their endurance, responsiveness, and their velocity in self-correction[2].

Cardiac surgery: the infinite quest. Part III - pediatric cardiac surgery: a discipline on its own / Cirurgia cardíaca: a busca infinita. Parte III - cirurgia cardíaca pediátrica: uma disciplina por si só

ULTRAMINI-ABSTRACT: Although there are common grounds with adult cardiac surgery, it is important to understand the differences in the business plan, paths, manpower, mindset, training, and infrastructure that are essential in those institutions where pediatric cardiac surgery can and should be performed. Time to start thinking, it is not what we can do, but should we do it?.