Right ventricular outflow tract stenting in the management of tetralogy of Fallot with pulmonary artery hypoplasia and major aortopulmonary collaterals

2020 ◽  
pp. 41-56
Author(s):  
Gemma Penford ◽  
Oliver Stumper
Keyword(s):  
Blood Flow ◽  
Pulmonary Blood Flow ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Fallot's Tetralogy ◽  
Operative Care ◽  
Key Points ◽  
Fallot’S Tetralogy ◽  
Surgical Options ◽  
Aortopulmonary Collaterals

Fallot’s tetralogy is the most common cyanotic congenital heart lesion. It is a term that encompasses a spectrum of morphologies, all emerging from the fundamental feature of anterior deviation of the outlet septum and associated abnormalities of pulmonary blood flow. This case follows the journey of a patient with severe Fallot’s tetralogy and multifocal pulmonary blood flow from the neonatal period through to his post-operative period. The case explores key points in the assessment of these patients, addressing the medical, interventional, and surgical options for neonatal cyanosis, and then goes on to discuss common issues and pitfalls surrounding peri-operative care.

Systemic hypertension in an infant with unrepaired tetralogy of Fallot: case report

2012 ◽  
Vol 23 (5) ◽  
pp. 746-748
Author(s):  
Michael Khoury ◽  
Michael Kallile ◽  
Joseph May ◽  
Rajesh Punn
Keyword(s):  
Blood Flow ◽  
Case Report ◽  
Tetralogy Of Fallot ◽  
Pulmonary Blood Flow ◽  
Pulmonary Valve ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Flow Dynamics ◽  
Systemic Hypertension ◽  
Pulmonary Valve Stenosis

AbstractPatients with severe right ventricular outflow tract obstruction in tetralogy of Fallot typically have right-to-left shunting, resulting in low pulmonary blood flow and cyanosis. Here we present the case of an infant with tetralogy of Fallot and severe pulmonary valve stenosis, complicated by systemic hypertension, the presence of which altered flow dynamics and possibly prevented cyanosis.

Effect of Accessory Pulmonary Blood Flow on Survival After the Bidirectional Glenn Procedure

Circulation ◽  
1999 ◽  
Vol 100 (suppl_2) ◽  
Author(s):  
Richard D. Mainwaring ◽  
John J. Lamberti ◽  
Karen Uzark ◽  
Robert L. Spicer ◽  
Mark W. Cocalis ◽  
...  
Keyword(s):  
Blood Flow ◽  
Mortality Rate ◽  
Pulmonary Blood Flow ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Right Ventricular Outflow Tract ◽  
Bidirectional Glenn ◽  
Actuarial Analysis ◽  
Bidirectional Glenn Procedure ◽  
Glenn Procedure

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.

Redistribution of myocardial fiber strain and blood flow by asynchronous activation

1990 ◽  
Vol 259 (2) ◽  
pp. H300-H308 ◽  
Author(s):  
F. W. Prinzen ◽  
C. H. Augustijn ◽  
T. Arts ◽  
M. A. Allessie ◽  
R. S. Reneman
Keyword(s):  
Blood Flow ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Left Ventricular Wall ◽  
Electrical Activation ◽  
Ventricular Wall ◽  
Activation Time ◽  
The Right ◽  
Ejection Phase

Hearts of 11 anesthetized open-chest dogs were paced from the right atrium (RA), right ventricular outflow tract (RVOT), and left ventricular apex (LVA). Maps of the sequence of electrical activation (192 electrodes), fiber strain (video technique), and blood flow (microsphere technique) in the epicardial layers were obtained from a 15- to 20-cm2 area of the anterior left ventricular wall. Electrical asynchrony in this area was 10 +/- 5 (RA), 52 +/- 12 (RVOT), and 30 +/- 16 ms (LVA, mean +/- SD, P less than 0.05 for RVOT and LVA compared with RA). Epicardial fiber strain during the ejection phase was uniformly distributed during RA pacing. However, during ventricular pacing it ranged from 13 +/- 33% (RVOT) and 23 +/- 29% (LVA) of the value during RA pacing in early-activated regions to 268 +/- 127% (RVOT) and 250 +/- 130% (LVA) of this value in late-activated regions. Epicardial blood flow ranged from 81 +/- 22% (RVOT) and 79 +/- 23% (LVA) in early-activated regions to 142 +/- 42% (RVOT) and 126 +/- 22% (LVA) in late activated regions. In all above values P less than 0.05 compared with RA. During RVOT pacing, gradients of epicardial electrical activation time, fiber strain, and blood flow pointed in the same direction. Compared with RVOT pacing, during LVA pacing all gradients were opposite in direction, and the gradients of electrical activation time and blood flow appeared to be smaller. These results indicate that timing of electrical activation is an important determinant for the distribution of fiber strain and blood flow in the left ventricular wall.

Abstract 4567: Functional Intraoperative Pulmonary Blood Flow Study is a Sensitive Predictor of Right Ventricular Pressure and Successful VSD Closure for Following Complete Uniforcalization in Patients with Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Osami Honjo ◽  
Osman O Al-Radi ◽  
Cathy MacDonald ◽  
Lisa Davey ◽  
Christopher A Caldarone ◽  
...  
Keyword(s):  
Blood Flow ◽  
Ventricular Septal Defect ◽  
Pulmonary Blood Flow ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Systolic Pressure ◽  
Pulmonary Flow ◽  
Aortopulmonary Collaterals ◽  
Major Aortopulmonary Collaterals ◽  
Flow Study

OBJECTIVE: We hypothesized mean pulmonary artery (PA) pressure obtained from an intraoperative pulmonary flow study would better predict the ability to close the ventricular septal defect (VSD) and better predict postoperative right ventricular systolic pressure (RSVP) than classic anatomical parameters after complete uniforcalization in patients with pulmonary atresia, VSD, and major aortopulmonary collaterals. METHODS: Sixteen consecutive patients (median, 11 mo, range 2 mo – 16 yrs) underwent one-stage (75%) or staged (25%) uniforcalization between 1/03 and 8/07. Intraoperative functional pulmonary blood flow study was achieved by inserting an arterial cannula in a reconstructed central PA. Flow was increased to 2.5 L/min/m2 while measuring PA pressure. RVSP and systemic systolic pressure (SBP) were recorded after VSD closure. Total neopulmonary artery index (TNPAI) (MAPCA + native PA index), total incorporated pulmonary vascular segments, and pulmonary segment artery ratio (PSAR) (ratio of incorporated segment to 18) were analyzed. Spearman rank correlation and area under the receiver operator characteristics curve (ROC-AUC) were used. RESULTS: The mean PA pressure on flow study was 21.8+/−6.2 mmHg (range, 11–31). Three patients had a pressure of > 30 mmHg. The VSD was closed in 14 (87%). One patient with flow study mean PA pressure of 25 mmHg had suprasystemic RVSP and underwent intraoperative VSD fenestration. One with a flow study mean PA pressure of 30 mmHg had a prospective fenestrated VSD patch placed. There is a weak negative correlation between TNPAI and the flow study PA pressure (rho=−0.4, p=0.12). The flow study mean PA pressure was correlated with post-repair RVSP (rho=0.72, p=0.0027), and with RVSP/SBP ratio (rho=0.67, p=0.0063). TNPAI, total incorporated segments, and PSAR were not correlated with the postoperative RVSP or RVSP/SBP ratio. Flow study mean PA pressure had the highest sensitivity in predicting VSD closure: ROC-AUC (0.82) vs. TNPAI (0.46), pulmonary segment (0.64), and PSAR (0.64). CONCLUSIONS: Intraoperative pulmonary flow study predicted the ability of VSD closure better than total incorporated segments, TNPAI, and PSAR. Flow study mean PA pressure highly correlated with postoperative RVSP and RVSP/SBP ratio.

Late replacement of the pulmonary valve: when and what type of valve?

2005 ◽  
Vol 15 (S1) ◽  
pp. 58-63 ◽  
Author(s):  
James A. Quintessenza ◽  
Jeffrey P. Jacobs ◽  
Paul J. Chai ◽  
Victor O. Morell ◽  
Jorge M. Giroud ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Pulmonary Insufficiency ◽  
Operative Strategy ◽  
Surgical Options ◽  
The Right

After repair of tetralogy of Fallot, many patients present in need of reoperative surgical reconstruction of the right ventricular outflow tract. The predominant physiologic lesion is pulmonary insufficiency, but there may also be varying degrees of obstruction of the right ventricular outflow tract. In the past, it has been felt that patients tolerate pulmonary insufficiency reasonably well. In some patients, however, the long-term effects of pulmonary insufficiency and subsequent right ventricular dilation and dysfunction are associated with poor exercise tolerance and increased incidence of arrhythmias and sudden death.1,2 Numerous studies support replacement of the pulmonary valve as treatment for pulmonary insufficiency in order to improve performance, optimize hemodynamics, and better control arrhythmias.3–10 The indications for reconstruction of the right ventricular outflow tract in this setting, nonetheless, as well as the operative strategy, continue to evolve. There are multiple surgical options for replacement of the pulmonary valve for these patients, including aortic and pulmonary homografts, stented and stentless porcine valves, porcine valved conduits, bovine jugular venous conduits, and even mechanical valves and mechanical valved conduits.11–32 It was a less than ideal experience with these currently available options that stimulated our interest into employing alternative materials and techniques. Favorable experimental and clinical experience with valves made of a polytetrafluoroethylene monoleaflet33–36 encouraged us to consider a new method of reconstruction with this material, using a bifoliate polytetrafluoroethylene valve. In this work, we review our indications for replacement of the pulmonary valve after repair of tetralogy of Fallot, the surgical options available, and our experience reconstructing the right ventricular outflow tract with a new surgically created bifoliate polytetrafluoroethylene valve.

Differential Alveolar and Systemic Oxygenation during Preterm Resuscitation with 100% Oxygen during Delayed Cord Clamping

2021 ◽  
Author(s):  
Satyan Lakshminrusimha ◽  
Payam Vali ◽  
Praveen Chandrasekharan ◽  
Wade Rich ◽  
Anup Katheria
Keyword(s):  
Blood Flow ◽  
Airway Pressure ◽  
Intraventricular Hemorrhage ◽  
Pulmonary Blood Flow ◽  
Venous Return ◽  
Positive Airway Pressure ◽  
Delayed Cord Clamping ◽  
Human Infants ◽  
Cord Clamping ◽  
Key Points

Objective Delayed cord clamping (DCC) and 21 to 30% O2 resuscitation is recommended for preterm infants but is commonly associated with low pulmonary blood flow (Qp) and hypoxia. 100% O2 supplementation during DCC for 60 seconds followed by 30% O2 may increase Qp and oxygen saturation (SpO2). Study Design Preterm lambs (125–127 days of gestation) were resuscitated with 100% O2 with immediate cord clamping (ICC, n = 7) or ICC + 30% O2, and titrated to target SpO2 (n = 7) or DCC + 100% O2 for 60 seconds, which followed by cord clamping and 30% O2 titration (n = 7). Seven preterm (23–27 weeks of gestation) human infants received continuous positive airway pressure (CPAP) + 100% O2 for 60 seconds during DCC, cord clamping, and 30% O2 supplementation after cord clamping. Results Preterm lambs in the ICC + 100% O2 group resulted in PaO2 (77 ± 25 mmHg), SpO2 (77 ± 11%), and Qp (27 ± 9 mL/kg/min) at 60 seconds. ICC + 30% O2 led to low Qp (14 ± 3 mL/kg/min), low SpO2 (43 ± 26%), and PaO2 (19 ± 7 mmHg). DCC + 100% O2 led to similar Qp (28 ± 6 mL/kg/min) as ICC + 100% O2 with lower PaO2. In human infants, DCC + CPAP with 100% O2 for 60 seconds, which followed by weaning to 30% resulted in SpO2 of 92 ± 11% with all infants >80% at 5 minutes with 100% survival without severe intraventricular hemorrhage. Conclusion DCC + 100% O2 for 60 seconds increased Qp probably due to transient alveolar hyperoxia with systemic normoxia due to “dilution” by umbilical venous return. Larger translational and clinical studies are warranted to confirm these findings. Key Points

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