Results of extracardiac conduit total cavopulmonary connection in 500 patients

Abstract

The early to mid-term outcomes of post-EC-TCPC patients managed with individualized pharmacotherapy were excellent, with low mortality and morbidity rates; however, development of late-occurring morbidities specific to Fontan physiology, including exercise intolerance and liver disease, must be carefully monitored during the long-term follow-up.

Noninvasive Assessment of Pulmonary Artery Pressure in Patients with Extracardiac Conduit Total Cavopulmonary Connection

“…CHARM study indicated PAP C 17 mmHg as one of the ''important hemodynamic variables associated with adverse outcomes'' in catheterization of single ventricle [16]. This cutoff point is more compatible with our situation in a developing country, although it is higher than the cutoff point of 10 mmHg which has been reported by Nakano et al [14]. It is worthy to note that the equations, concept of correlations between IVC-CI and mean PAP and the clinical usage of these indexes for quantitative long-term follow-up of an individual patient are not significantly dependent on this cutoff point.…”

“…However, lack of definite and accurate noninvasive methods for quantitative estimation of PAP, collateral evaluation and difficulty in obtaining good quality echocardiographic images in adults with congenital heart disease is challenging areas in TCPC patients. Cardiac catheterization is the gold standard method for assessment of TCPC pathway, PAP assessment and sometimes intervention as the next step [14]. Some experts routinely perform angiography in TCPC patients, but controversy over the need of angiography in TCPC patients persists due to the invasive nature of the procedure, ionizing radiation and risk of complications, such as emboli [5].…”

“…Kaulitz et al recommended cardiac catheterization in a regular plan for postoperative evaluation of TCPC and reported 94 % and 82 % intervention freedom after 5 and 10 years follow-up [15]. Nakano et al, in a large report of Japanese TCPC patient follow-ups, present a predefined regular cardiac catheterization protocol (in an interval of 6 months after TCPC and then in 6, 12, 18 year old ones); the results showed excellent TCPC outcome with regard to mortality, morbidity and even very impressive PAP [14]. Studies with a lower mean PAP have more catheterization freedom [14].…”

“…Nakano et al, in a large report of Japanese TCPC patient follow-ups, present a predefined regular cardiac catheterization protocol (in an interval of 6 months after TCPC and then in 6, 12, 18 year old ones); the results showed excellent TCPC outcome with regard to mortality, morbidity and even very impressive PAP [14]. Studies with a lower mean PAP have more catheterization freedom [14]. However, due to delay in diagnosis and referral, our study population was in the age range of 5.5-32 years at the time of completion of TCPC and had a mean PAP in the range of 8-22 mmHg (only 3 patients had PAP \ 10 mmHg) during the present study.…”

Avaliação cardiovascular, pulmonar e musculoesquelética em pacientes com fisiologia univentricular no período pós-operatório tardio da cirurgia de Fontan

“…Considerando os avanços, tanto em decorrência de melhorias na técnica cirúrgica, como no manejo peri-operatório, a sobrevida desta população é bastante satisfatória, ao redor de 85% de sobrevida em vinte anos, nos melhores centros do mundiais (9,(18)(19)(20).…”