Modified fontan operation in functionally univentricular hearts: Preoperative risk factors and intermediate results

Kaulitz, Renate; Ziemer, Gerhard; Luhmer, I; Kallfelz, H. C.

doi:10.1016/s0022-5223(96)70049-1

Cited by 89 publications

(47 citation statements)

References 27 publications

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“…1,9 -11 The reasons for deterioration are multifactorial, but a low pulmonary vascular resistance remains an important prognostic factor for a good functional result after the Fontan operation. 12 NO has been used to treat pulmonary endothelial dysfunction resulting from cardiopulmonary bypass after Fontan operation. 5,13 However, to our knowledge, there are no studies examining the effect of inhaled NO on pulmonary vascular resistance late after Fontan-type operations.…”

Section: Discussionmentioning

confidence: 99%

Basal Pulmonary Vascular Resistance and Nitric Oxide Responsiveness Late After Fontan-Type Operation

et al. 2003

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Background-The pulsatile nature of pulmonary blood flow is important for shear stress-mediated release of endothelium-derived nitric oxide (NO) and lowering pulmonary vascular resistance (PVR) by passive recruitment of capillaries. Normal pulsatile flow is lost or markedly attenuated after Fontan-type operations, but to date, there are no data on basal pulmonary vascular resistance and its responsiveness to exogenous NO at late follow-up in these patients. Methods and Results-We measured indexed PVR (PVRI) using Fick principle to calculate pulmonary blood flow, with respiratory mass spectrometry to measure oxygen consumption, in 15 patients (median age, 12 years; range, 7 to 17 years; 12 male, 3 female) at a median of 9 years after a Fontan-type operation (6 atriopulmonary connections, 7 lateral tunnels, 2 extracardiac conduits). The basal PVRI was 2.11Ϯ0.79 Wood unit (WU) times m 2 (meanϮSD) and showed a significant reduction to 1.61Ϯ0.48 (Pϭ0.016) after 20 ppm of NO for 10 minutes. The patients with nonpulsatile group in the pulmonary circulation dropped the PVRI from 2.18Ϯ0.34 to 1.82Ϯ0.55 (PϽ0.05) after NO inhalation. Conclusions-PVR falls with exogenous NO late after Fontan-type operation. These data suggest pulmonary endothelial dysfunction, related in some part to lack of pulsatility in the pulmonary circulation because of altered flow characteristics. Therapeutic strategies to enhance pulmonary endothelial NO release may have a role in these patients.

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Section: Discussionmentioning

confidence: 99%

Basal Pulmonary Vascular Resistance and Nitric Oxide Responsiveness Late After Fontan-Type Operation

et al. 2003

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“…33 Fontan patients residing at moderate altitude present with worse prognosis, presumably related to an increase in pulmonary vascular resistance. 34 Khambadkone et al 3 showed that pulmonary vascular resistance was elevated late after the Fontan operation and may fall in response to inhaled NO.…”

Section: Effect Of Sildenafil On Exercise Hemodynamicsmentioning

confidence: 99%

Sildenafil Improves Exercise Hemodynamics in Fontan Patients

Bruaene

Gerche

Claessen

et al. 2014

Circ: Cardiovascular Imaging

126

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Sildenafil targets the endothelial NO pathway by inhibiting phosphodiesterase-5, increasing cGMP, and inducing pulmonary vasodilation.7 Giardini et al 8 demonstrated an improvement in peak oxygen consumption and CI after a single oral Background-Patients with Fontan circulation have reduced exercise capacity. The absence of a presystemic pump may limit flow through the pulmonary circulation, restricting ventricular filling and cardiac output. We evaluated exercise hemodynamics and the effect of sildenafil on exercise hemodynamics in Fontan patients. Methods and Results-Ten Fontan patients (6 men, 20±4 years) underwent cardiac magnetic resonance imaging at rest and during supine bicycle exercise before and after sildenafil. Systemic ventricular volumes were obtained at rest and during low-(34±15 W), moderate-(69±29 W), and high-intensity (97±36 W) exercise using an ungated, free-breathing cardiac magnetic resonance sequence and analyzed correcting for cardiac phase and respiratory translation. Radial and pulmonary artery pressures and cGMP were measured. Before sildenafil, cardiac index increased throughout exercise (4.0±0.9, 5.9±1.1, 7.0±1.6, 7.4±1.7 L/(min·m 2 ); P<0.0001) with 106±49% increase in heart rate. Stroke volume index (P=0.015) and end-diastolic volume index (P=0.001) decreased during exercise. End-systolic volume index remained unchanged (P=0.8). Total pulmonary resistance index (P=0.005) increased, whereas systemic vascular resistance index decreased during exercise (P<0.0001). Sildenafil increased cardiac index (P<0.0001) and stroke volume index (P=0.003), especially at high-intensity exercise (interaction P=0.004 and P=0.003, respectively). Systemic vascular resistance index was reduced (P<0.0001-interaction P=0.1), whereas total pulmonary resistance index was reduced at rest and reduced further during exercise (P=0.008-interaction P=0.029). cGMP remained unchanged before sildenafil (P=0.9), whereas it increased significantly after sildenafil (P=0.019). Conclusions-In Fontan patients, sildenafil improved cardiac index during exercise with a decrease in total pulmonary resistance index and an increase in stroke volume index. This implies that pulmonary vasculature represents a physiological limitation, which can be attenuated by sildenafil, the clinical significance of which warrants further study. (Circ Cardiovasc Imaging. 2014;7:265-273.)

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“…Currently, there are two techniques of Fontan procedure: intra-atrial LT [20][21][22] and extracardiac conduit [23,24]. Intra-atrial tunnel is associated with an increased risk of postoperative atrial rhythm disorders due to long suture lines within the atrium.…”

Section: Discussionmentioning

confidence: 99%

Results of the Fontan operation with no early mortality in 248 consecutive patients

2017

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A b s t r a c t Background:The Fontan operation has undergone several modifications and today is the primary way to treat a broad spectrum of congenital heart defects. Aim:The purpose of this study is to present the results of treatment of children with a single ventricle operated by the same surgical team and managed according to a uniform strategy. Methods:In the years 2007-2015, in 248 children aged 3.7 ± 2.6 years and weighing 14.6 ± 6.1 kg with a single ventricle, Fontan surgery was performed. In 56 (22.6%) children surgery was based on the creation of an intra-atrial lateral tunnel, and in 192 (77.4%) patients extracardiac modification was performed. In most patients, the operation was carried out with the normothermic cardiopulmonary bypass, on a "beating heart" without aortic cross-clamp. The average cardiopulmonary bypass time was 53.9 ± 23.9 min. The most common indication for surgery was hypoplastic left heart syndrome (53.6%). All patients with a single ventricle referred to our hospital for the Fontan procedure were enrolled into the surgery programme.Results: All patients survived the operation and were discharged home. Thirty-six (14.5%) patients were extubated in the operating room, in other patients the mean duration of the mechanical ventilation was 9.7 ± 16.1 h (median 7 h). The average time of hospitalisation in the whole study group was 17.5 ± 18.5 days (median 15 days). After surgery, in four children transient seizures occurred, and three patients had an ischaemic stroke. Conclusions:Developing and obeying a fixed perioperative protocol is crucial for low mortality and small number of complications after Fontan operation.

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Modified fontan operation in functionally univentricular hearts: Preoperative risk factors and intermediate results

Abstract: In a high proportion of hearts with complex univentricular anatomy, associated anomalies, and borderline hemodynamics, the Fontan operation can be performed as a total cavopulmonary anastomosis with acceptable intermediate postoperative morbidity and hemodynamic results.

Cited by 89 publications

References 27 publications

Basal Pulmonary Vascular Resistance and Nitric Oxide Responsiveness Late After Fontan-Type Operation

Basal Pulmonary Vascular Resistance and Nitric Oxide Responsiveness Late After Fontan-Type Operation

Sildenafil Improves Exercise Hemodynamics in Fontan Patients

Results of the Fontan operation with no early mortality in 248 consecutive patients

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