Maldistribution of pulmonary blood flow in patients after the Fontan operation is associated with worse exercise capacity

Background —The bidirectional Glenn procedure (BDG) is used in the staged surgical management of patients with a functional single ventricle. Controversy exists regarding whether accessory pulmonary blood flow (APBF) should be left at the time of BDG to augment systemic saturation or be eliminated to reduce volume load of the ventricle. The present study was a retrospective review of patients undergoing BDG that was conducted to assess the influence of APBF on survival rates. Methods and Results —From 1986 through 1998, 149 patients have undergone BDG at our institution. Ninety-three patients had elimination of all sources of APBF, whereas 56 patients had either a shunt or a patent right ventricular outflow tract intentionally left in place to augment the pulmonary blood flow provided by the BDG. The operative mortality rate was 2.2% without APBF and 5.4% with APBF. The late mortality rate was 4.4% without APBF and 15.1% with APBF. Actuarial analysis demonstrates a divergence of the Kaplan-Meier curves in favor of patients in whom APBF was eliminated ( P <0.02). One hundred seven patients have subsequently undergone completion of their Fontan operation, so the actuarial analysis includes the operative risk of this second operation. Conclusions —The results suggest that the elimination of APBF at the time of BDG may confer a long-term advantage for patients with a functional single ventricle.

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Ventilation with external high frequency oscillation around a negative baseline increases pulmonary blood flow after the Fontan operation

Cardiology in the Young ◽

10.1017/s1047951100001049 ◽

1992 ◽

Vol 2 (3) ◽

pp. 277-280 ◽

Cited By ~ 14

Author(s):

Daniel J. Penny ◽

Zamir Hayek ◽

Peter Rawle ◽

Michael L. Rigby ◽

Andrew N. Redington

Keyword(s):

Blood Flow ◽

Negative Pressure ◽

High Frequency ◽

Pulmonary Blood Flow ◽

Positive Pressure Ventilation ◽

Fontan Operation ◽

Intermittent Positive Pressure Ventilation ◽

High Frequency Oscillation ◽

Positive Pressure ◽

Frequency Oscillation

AbstractIn this prospective study, pulmonary blood flow was measured using transesophageal Doppler echocardiography to assess whether ventilation by means of external high frequency oscillation around a negative pressure baseline can increase pulmonary blood flow, compared to intermittent positive pressure ventilation, in five patients after the Fontan operation. Pulmonary blood flow was measured when patients were ventilated by means of intermittent positive pressure ventilation and again during equivalent negative pressure ventilation using the external oscillatory technique. When compared to that with intermittent positive pressure ventilation, ventilation using external high frequency oscillation increased pulmonary blood flow by 116 ±61.5% (p=0.013). These results show that ventilation using an external oscillatory device with a mean negative chamber pressure may provide hemodynamic advantages in patients requiring assisted ventilation after the Fontan operation.

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Abstract 17326: The Effect of Maintenance of Antegrade Pulmonary Blood Flow Upon Bidirectional Glenn on Pre- and Post-Fontan Outcomes

Circulation ◽

10.1161/circ.138.suppl_1.17326 ◽

2018 ◽

Vol 138 (Suppl_1) ◽

Author(s):

Jae Suk Baek ◽

Chun Soo Park ◽

Eun Seok Choi ◽

Bo Sang Kwon ◽

Tae-Jin Yun ◽

...

Keyword(s):

Blood Flow ◽

Oxygen Saturation ◽

Pulmonary Blood Flow ◽

Fontan Operation ◽

Advantages And Disadvantages ◽

Bidirectional Glenn ◽

Pulmonary Arterial ◽

Group 2 ◽

The Impact ◽

Group 1

Introduction: Maintaining pulsatility at the time of bidirectional Glenn (BDG) has theoretical advantages and disadvantages. The practice is diverse throughout the centers and individual surgeons without clear rationale behind its application. We sought to investigate the impact of antegrade pulmonary blood flow on pre- and post-Fontan outcomes. Methods: From 2003 through June 2014, among 237 patients who underwent BDG as an interim palliation for functional single ventricle (FSV), patients with preexisting pulmonary blood flow from the ventricle before BDG were included and patients undergoing Kawashima operation or having history of Norwood operation or bilateral pulmonary arterial band were excluded (n=151). Patients were grouped by their presence or absence of antegrade pulmonary blood flow following BDG: group 1 (pulsatile) (n=73) or group 2 (non-pulsatile) (n=78). The pre- and post-Fontan outcomes were compared between two groups. Results: Age and weight at BDG were 7 months and 7.8kg. Oxygen saturation was higher in group 1 (84±4%) compared to group 2 (82±4%) before Fontan (p<0.001). The level of brain natriuretic peptide (BNP) was similar before Fontan (p=0.966). In pre-Fontan cardiac catheterization, mean pulmonary arterial pressure was similar (Group 1: 11±4mmHg vs. Group 2: 10±4mmHg, p=0.146), McGoon ratio seemed higher in group 1(2.6±0.6) compared to group 2(2.3±0.4) (p=0.057), and pulmonary vascular resistance was lower in group 1(1.3±0.7) compared to group 2(1.6±0.7)(p=0.038). A total of 136 patients (90%) underwent Fontan operation. Duration of stay in the intensive care unit (p=0.766), stay in hospital (p=0.142), and indwelling chest tube (p=0.128) was not different between groups. Overall survival after BDG was better in group 2 (p=0.046) [Figure]. In multivariable analysis, maintenance of pulsatility was identified as a risk factor for survival after BDG (p=0.012, HR 4.1 [CI 1.4-12.4]). Conclusions: Maintaining pulsatility at the time of BDG in FSV might be beneficial for pulmonary arterial growth and oxygen saturation without increasing BNP. However, such beneficial effect did not have a positive effect on subsequent Fontan outcome. Attention must be paid to the negative effect of pulsatility on overall post-BDG survival.

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A Hemi Fontan Operation Performed by an Engineer: Considerations on Virtual Surgery

Volume 1B: Extremity; Fluid Mechanics; Gait; Growth, Remodeling, and Repair; Heart Valves; Injury Biomechanics; Mechanotransduction and Sub-Cellular Biophysics; MultiScale Biotransport; Muscle, Tendon and Ligament; Musculoskeletal Devices; Multiscale Mechanics; Thermal Medicine; Ocular Biomechanics; Pediatric Hemodynamics; Pericellular Phenomena; Tissue Mechanics; Biotransport Design and Devices; Spine; Stent Device Hemodynamics; Vascular Solid Mechanics; Student Paper and Design Competitions ◽

10.1115/sbc2013-14451 ◽

2013 ◽

Author(s):

Giovanni Biglino ◽

Ethan Kung ◽

Adam Dorfman ◽

Andrew M. Taylor ◽

Edward Bove ◽

...

Keyword(s):

Blood Flow ◽

Pulmonary Blood Flow ◽

Fontan Operation ◽

Hybrid Approach ◽

Pulmonary Arteries ◽

Virtual Surgery ◽

Extracardiac Conduit ◽

Lateral Tunnel ◽

The Right ◽

Cavopulmonary Connection

Single ventricle circulation, characterized at birth by a rudimentary or absent left or right ventricle, presents a challenging and life-threatening physiological scenario. Surgical palliation aims to restore the balance between systemic and pulmonary blood flow and is staged, each of the three stages presenting the surgeon with different options: - Stage 1 (Norwood procedure) involves different types of shunting to source pulmonary blood flow, or recently a hybrid approach [1]; - Stage 2 can involve a superior cavopulmonary connection (Glenn operation) or patching between the right atrium and the pulmonary arteries (Hemi Fontan operation [2]); - Stage 3 involves a total cavopulmonary connection with extracardiac conduit or lateral tunnel, or with novel alternatives such as the Y-graft [3].

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Transcatheter closure of antegrade pulmonary blood flow with Amplatzer muscular VSD occluder after Fontan operation

Anadolu Kardiyoloji Dergisi/The Anatolian Journal of Cardiology ◽

10.5152/akd.2014.5560 ◽

2014 ◽

Vol 14 (6) ◽

pp. 565-565 ◽

Cited By ~ 1

Author(s):

Tevfik Karagoz ◽

Mustafa Gulgun ◽

Metin Demircin ◽

Hayrettin Hakan Aykan ◽

Alper Akin

Keyword(s):

Blood Flow ◽

Pulmonary Blood Flow ◽

Transcatheter Closure ◽

Fontan Operation ◽

Muscular Vsd

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Transcatheter closure of antegrade pulmonary blood flow with Amplatzer septal occluder after Fontan operation

Anadolu Kardiyoloji Dergisi/The Anatolian Journal of Cardiology ◽

10.5152/akd.2015.6045 ◽

2015 ◽

Vol 15 (2) ◽

pp. 165-166

Author(s):

Alper Guzeltas ◽

Ibrahim Cansaran Tanidir ◽

Murat Saygi

Keyword(s):

Blood Flow ◽

Pulmonary Blood Flow ◽

Transcatheter Closure ◽

Fontan Operation ◽

Amplatzer Septal Occluder

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Impaired Exercise Tolerance in Repaired Tetralogy of Fallot Is Associated With Impaired Biventricular Contractile Reserve: An Exercise-Stress Real-Time Cardiovascular Magnetic Resonance Study

Circulation Cardiovascular Imaging ◽

10.1161/circimaging.120.011823 ◽

2021 ◽

Author(s):

Michael Steinmetz ◽

Thomas Stümpfig ◽

Matthias Seehase ◽

Andreas Schuster ◽

Johannes Kowallick ◽

...

Keyword(s):

Blood Flow ◽

Cardiovascular Magnetic Resonance ◽

Magnetic Resonance ◽

Real Time ◽

Exercise Capacity ◽

Body Surface ◽

Pulmonary Blood Flow ◽

Pulmonary Valve ◽

Lv Function ◽

End Diastolic Volume

Background: Correction of tetralogy of Fallot (cTOF) often results in pulmonary valve pathology and right ventricular (RV) dysfunction. Reduced exercise capacity in cTOF patients cannot be explained by these findings alone. We aimed to explore why cTOF patients exhibit impaired exercise capacity with the aid of a comprehensive cardiopulmonary exercise testing (CPET) and real-time cardiovascular magnetic resonance exercise testing (CMR-ET) protocol. Methods: Three cTOF patients and 35 matched healthy controls underwent CPET and CMR-ET in a prospective case-control study. Real-time steady-state free precession cine and phase-contrast sequences were obtained during incremental supine in-scanner cycling at 50, 70, and 90 W. RV and left ventricle (LV) volumes and pulmonary blood flow (Qp) were calculated. Differences of CPET and CMR-ET between cTOF versus controls and correlations between CPET and CMR-ET parameters in cTOF were evaluated statistically for all CMR exercise levels using Mann-Whitney U and Spearman rank-order correlation tests. Results: CPET capacity was significantly lower in cTOF than in controls. cTOF patients exhibited not only significantly reduced Qp and RV function but also lower LV function on CMR-ET. Higher CPET values in cTOF correlated with higher Qp (Qp 90 W versus carbon dioxide ventilatory equivalent %: R =−0.519, P <0.05), higher LV–end-diastolic volume indexed to body surface area (LV–end-diastolic volume indexed to body surface area at 50 W versus oxygen uptake in % at maximum exercise on CPET R =0.452, P <0.05), and change in LV ejection fraction (EF; LV-EF at 90 W versus Watt %: r =−0.463, P <0.05). No correlation was found with regard to RV-EF. Significant RV-LV interaction was observed during CMR-ET (RV-EF versus LV-EF at 50 W and 70 W: r =0.66, P <0.02 and r =0.52, P <0.05, respectively). Conclusions: Impaired exercise capacity in cTOF resulted from a reduction in not only RV, but also LV function. cTOF with good exercise capacity on CPET demonstrated higher LV reserve and pulmonary blood flow during incremental CMR-ET. Apart from RV parameters, CMR-ET–derived LV function could be a valuable tool to stratify cTOF patients for pulmonary valve replacement.

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Outcomes of Primary Bidirectional Glenn in Children with Single Ventricle Physiology and Increased Pulmonary Blood Flow

The Heart Surgery Forum ◽

10.1532/hsf.3299 ◽

2020 ◽

Vol 23 (6) ◽

pp. E850-E856

Author(s):

Ahmed Mohamed Dohain ◽

Muhammed A. Mashat ◽

Ahmed M.A Al-Mojaddidi ◽

Mohamed E. Abdelmotaleb ◽

Abdulla A. Mashat ◽

...

Keyword(s):

Blood Flow ◽

Pulmonary Blood Flow ◽

Single Ventricle ◽

Fontan Operation ◽

Length Of Hospital Stay ◽

University Hospital ◽

Second Stage ◽

Bidirectional Glenn ◽

Significant Difference ◽

King Abdulaziz University

Background: We reported our experience in managing patients with single ventricle (SV) physiology and increased pulmonary blood flow (PBF), aiming to assess if it is feasible to proceed with primary Bidirectional Glenn (BDG) without a prior operation to limit PBF. Materials and methods: This is a retrospective study with 51 consecutive patients who underwent BDG operation as a primary operation or a second stage prior to the definitive Fontan operation at King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia between 2010 and 2018. Patients were categorized into two groups based on their PBF prior to the operation: Patients who had SV physiology and increased PBF (seven patients) vs. patients with SV physiology and restricted PBF (44 patients). Results: The median age for the increased PBF group was 9.9 months [interquartile range (IQR): 2-16.9 months], and the median age for the restricted PBF group was 15.3 months (IQR: 6.7-42.6 months). Although the length of hospital stay was longer in patients with increased PBF (P = 0.039), we couldn't find a statistically significant difference in early mortality, duration of mechanical ventilation, length of pleural drainage, and length of intensive care unit (ICU) stay between the groups. Conclusion: In our experience, we found that primary BDG could be done safely for patients having SV physiology and increased PBF with acceptable short-term outcomes. It might further reduce the morbidity and mortality for those patients by avoiding the risk of initial pulmonary artery banding or aortopulmonary shunts.

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Breathing training improves exercise capacity in patients with tetralogy of Fallot: a randomised trial

Heart ◽

10.1136/heartjnl-2020-318574 ◽

2021 ◽

pp. heartjnl-2020-318574

Author(s):

Julia Hock ◽

Julia Remmele ◽

Renate Oberhoffer ◽

Peter Ewert ◽

Alfred Hager

Keyword(s):

Blood Flow ◽

Tetralogy Of Fallot ◽

Exercise Capacity ◽

Pulmonary Blood Flow ◽

Randomised Trial ◽

Cardiopulmonary Exercise Testing ◽

Young Patients ◽

Control Group ◽

Lung Volumes ◽

Breathing Training

ObjectivePatients with tetralogy of Fallot (ToF) have limited pulmonary blood flow before corrective surgery and ongoing dysfunction of the pulmonary valve and right ventricle throughout life leading to lower exercise capacity and lung volumes in many patients. Inhalation training can increase lung volumes, improve pulmonary blood flow and consequently exercise capacity. This study tests whether home-based daily breathing training improves exercise capacity and lung volumes.MethodsFrom February 2017 to November 2018, 60 patients (14.7±4.8 years, 39% female) underwent spirometry (forced vital capacity (FVC); forced expiratory volume in 1 s (FEV1)), cardiopulmonary exercise testing (peak oxygen uptake (peak V˙O2)) and breathing excursion measurement. They were randomised into immediate breathing exercise or control group (CG) and re-examined after 6 months. The CG started their training afterwards and were re-examined after further 6 months. Patients trained with an inspiratory volume-oriented breathing device and were encouraged to exercise daily. The primary endpoint of this study was the change in peak V˙O2. Results are expressed as mean±SEM (multiple imputations).ResultsIn the first 6 months (intention to treat analysis), the training group showed a more favourable change in peak V˙O2 (Δ0.5±0.6 vs −2.3±0.9 mL/min/kg, p=0.011), FVC (Δ0.18±0.03 vs 0.08±0.03 L, p=0.036) and FEV1 (Δ0.14±0.03 vs −0.00±0.04 L, p=0.007). Including the delayed training data from the CG (n=54), this change in peak V˙O2 correlated with self-reported weekly training days (r=0.282, p=0.039).ConclusionsDaily inspiratory volume-oriented breathing training increases dynamic lung volumes and slows down deconditioning in peak V˙O2 in young patients with repaired ToF.

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