Fontan completion in reverse order out of necessity: secondary Glenn after primary extracardiac inferior cavopulmonary artery connection

2016 ◽  
Vol 27 (5) ◽  
pp. 925-928 ◽  
Author(s):  
Jannika Dodge-Khatami ◽  
Avichal Aggarwal ◽  
Mary B. Taylor ◽  
Douglas Maposa ◽  
Jorge D. Salazar ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Upper Body ◽  
Reverse Order ◽  
Venous Anatomy ◽  
Caval Vein ◽  
Single Ventricle Physiology ◽  
Superior Caval Vein ◽  
Good Outcome ◽  
Cavopulmonary Connection

AbstractThe primary extracardiac inferior cavopulmonary connection is an unusual novel palliation for single-ventricle physiology, which we first performed in the setting of unfavourable upper-body systemic venous anatomy for a standard bi-directional Glenn, and in lieu of leaving our patient with shunt-dependent physiology. After an initial 16-month satisfactory follow-up, increasing cyanosis led to the discovery of a veno-venous collateral that was coiled, but, more importantly, to impressive growth of a previously diminutive superior caval vein, which allowed us to perform completion Fontan with a good outcome. Performing the single-ventricle staging in a reverse manner, first from below with a primary inferior cavopulmonary connection, followed by Fontan completion from above with a standard superior caval vein bi-directional Glenn, is also possible when deemed necessary.

When the bi-directional Glenn is an unfavourable option: primary extracardiac inferior cavopulmonary connection as an alternative palliation

2015 ◽  
Vol 26 (7) ◽  
pp. 1247-1249 ◽  
Author(s):  
Ali Dodge-Khatami ◽  
Avichal Aggarwal ◽  
Mary B. Taylor ◽  
Douglas Maposa ◽  
Jorge D. Salazar
Keyword(s):  
Single Ventricle ◽  
Venous Return ◽  
Upper Body ◽  
Caval Vein ◽  
Extracardiac Conduit ◽  
Cavopulmonary Anastomosis ◽  
Operative Outcome ◽  
Vein Stenosis ◽  
Cavopulmonary Connection

AbstractThe superior cavopulmonary anastomosis – bi-directional Glenn – is the standard palliation for single ventricle physiology. When upper body systemic venous anatomic concerns such as superior caval vein stenosis, hypoplasia, or inadequate collateral tributaries are present, a Glenn may be precluded or have a high risk of poor outcome. A primary inferior cavopulmonary connection with an extracardiac conduit is an alternative palliation that provides a generous pathway for pulmonary blood flow, with the additional benefit of including hepatic venous return. We report a case of primary extracardiac inferior cavopulmonary connection in a patient unsuitable for Glenn, with successful post-operative outcome and early follow-up.

Does superior caval vein pressure impact head growth in Fontan circulation?

2016 ◽  
Vol 26 (7) ◽  
pp. 1327-1332
Author(s):  
Tina Trachsel ◽  
Christian Balmer ◽  
Håkan Wåhlander ◽  
Roland Weber ◽  
Hitendu Dave ◽  
...  
Keyword(s):  
Head Circumference ◽  
Single Ventricle ◽  
Communicating Hydrocephalus ◽  
Caval Vein ◽  
Single Ventricle Physiology ◽  
Superior Caval Vein ◽  
Cavopulmonary Anastomosis ◽  
Pressure Impact ◽  
By Catch ◽  
Bidirectional Cavopulmonary Anastomosis

AbstractBackgroundPatients with bidirectional cavopulmonary anastomosis have unphysiologically high superior caval vein pressure as it equals pulmonary artery pressure. Elevated superior caval vein pressure may cause communicating hydrocephalus and macrocephaly. This study analysed whether there exists an association between head circumference and superior caval vein pressure in patients with single ventricle physiology.MethodsWe carried out a retrospective analysis of infants undergoing Fontan completion at our institution from 2007 to 2013. Superior caval vein pressures were measured during routine catheterisation before bidirectional cavopulmonary anastomosis and Fontan completion as well as head circumference, adjusted to longitudinal age-dependent percentiles.ResultsWe included 74 infants in our study. Median ages at bidirectional cavopulmonary anastomosis and Fontan were 4.8 (1.6–12) and 27.9 (7–40.6) months, respectively. Head circumference showed significant growth from bidirectional cavopulmonary anastomosis until Fontan completion (7th (0–100th) versus 20th (0–100th) percentile). There was no correlation between superior caval vein pressure and head circumference before Fontan (R2=0.001). Children with lower differences in superior caval vein pressures between pre-bidirectional cavopulmonary anastomosis and pre-Fontan catheterisations showed increased growth of head circumference (R2=0.19).ConclusionsPatients with moderately elevated superior caval vein pressure associated with single ventricle physiology did not have a tendency to develop macrocephaly. There is no correlation between superior caval vein pressure before Fontan and head circumference, but between bidirectional cavopulmonary anastomosis and Fontan head circumference increases significantly. This may be explained by catch-up growth of head circumference in patients with more favourable haemodynamics and concomitant venous pressures in the lower range. Further studies with focus on high superior caval vein pressures are needed to exclude or prove a correlation.

An unexpected case of post-operative superior caval vein syndrome

2018 ◽  
Vol 28 (6) ◽  
pp. 879-881 ◽  
Author(s):  
Lynn Peng ◽  
Lisa Wise-Faberowski
Keyword(s):  
Unusual Case ◽  
Heart Surgery ◽  
Single Ventricle ◽  
Congenital Heart Surgery ◽  
Caval Vein ◽  
External Compression ◽  
Superior Caval Vein ◽  
Single Ventricle Palliation ◽  
Catheterisation Laboratory ◽  
Tetralogy Of Fallot Repair

AbstractSuperior caval vein obstruction in children after congenital heart surgery has been more associated with thrombosis formation as result of single-ventricle palliation, infection, indwelling devices/catheters, or external compression. Many of these patients will present to the cardiac catheterisation laboratory for evaluation and possible intervention. We present an unusual case of superior caval vein obstruction in a patient after Tetralogy of Fallot repair.

Angiotensin-converting enzyme inhibition and pre-superior cavopulmonary connection haemodynamics in infants with single-ventricle physiology

2021 ◽  
pp. 1-5
Author(s):  
Asim Al Balushi ◽  
Konstantin Averin ◽  
Daphne T. Hsu ◽  
Andrew S. Mackie
Keyword(s):  
Placebo Group ◽  
Single Ventricle ◽  
Group Versus ◽  
Angiotensin Converting Enzyme Inhibition ◽  
Flow Ratio ◽  
Single Ventricle Physiology ◽  
Converting Enzyme Inhibition ◽  
Secondary Outcomes ◽  
Cavopulmonary Connection ◽  
Enalapril Group

Abstract Introduction: Preliminary animal and human data suggest that angiotensin-converting enzyme inhibition has a role in pulmonary vascular remodelling. We sought to assess the effect of ACEi versus placebo on pulmonary artery pressure and transpulmonary gradient amongst infants undergoing single-ventricle palliation. Materials and methods: Using the publicly available Pediatric Heart Network Infant Single-Ventricle trial dataset, we compared mean PA pressure at pre-superior cavopulmonary connection catheterisation (primary outcome), transpulmonary gradient, pulmonary-to-systemic flow ratio, and post-SCPC oxygen saturation (secondary outcomes) in infants receiving enalapril versus placebo. Results: A total of 179 infants underwent pre-SCPC catheterisation, of which 85 (47%) received enalapril. There was no difference between the enalapril and placebo group in the primary and the secondary outcomes. Mean PA pressure in the enalapril group was 13.1 ± 2.9 compared to 13.7 ± 3.4 mmHg in the placebo group. The transpulmonary gradient was 6.7 ± 2.5 versus 6.9 ± 3.2 mmHg in the enalapril and placebo groups, respectively. The pulmonary-to-systemic flow ratio was 1.1 ± 0.5 in the enalapril group versus 1.0 ± 0.5 in the placebo group and the post-SCPC saturation was 83.1 ± 5.0% in the enalapril group versus 82.2 ± 5.3% in the placebo group. In the pre-specified subgroup analyses comparing enalapril and placebo according to ventricular morphology and shunt type, there was no difference in the primary and secondary outcomes. Conclusion: ACEi did not impact mean pulmonary artery pressure or transpulmonary gradient amongst infants with single-ventricle physiology prior to SCPC palliation.

scholarly journals Passive range of motion exercise to enhance growth in infants following the Norwood procedure: a safety and feasibility trial

2017 ◽  
Vol 27 (7) ◽  
pp. 1361-1368 ◽  
Author(s):  
Linda M. Lambert ◽  
Felicia L. Trachtenberg ◽  
Victoria L. Pemberton ◽  
Janine Wood ◽  
Shelley Andreas ◽  
...  
Keyword(s):  
Adverse Events ◽  
Range Of Motion ◽  
Single Ventricle ◽  
Vital Signs ◽  
Exercise Programme ◽  
Stage Ii ◽  
Z Score ◽  
Single Ventricle Physiology ◽  
Motion Exercise

AbstractObjectiveThe aim of this study was to evaluate the safety and feasibility of a passive range of motion exercise programme for infants with CHD.Study designThis non-randomised pilot study enrolled 20 neonates following Stage I palliation for single-ventricle physiology. Trained physical therapists administered standardised 15–20-minute passive range of motion protocol, for up to 21 days or until hospital discharge. Safety assessments included vital signs measured before, during, and after the exercise as well as adverse events recorded through the pre-Stage II follow-up. Feasibility was determined by the percent of days that >75% of the passive range of motion protocol was completed.ResultsA total of 20 infants were enrolled (70% males) for the present study. The median age at enrolment was 8 days (with a range from 5 to 23), with a median start of intervention at postoperative day 4 (with a range from 2 to 12). The median hospital length of stay following surgery was 15 days (with a range from 9 to 131), with an average of 13.4 (with a range from 3 to 21) in-hospital days per patient. Completion of >75% of the protocol was achieved on 88% of eligible days. Of 11 adverse events reported in six patients, 10 were expected with one determined to be possibly related to the study intervention. There were no clinically significant changes in vital signs. At pre-Stage II follow-up, weight-for-age z-score (−0.84±1.20) and length-for-age z-score (−0.83±1.31) were higher compared with historical controls from two earlier trials.ConclusionA passive range of motion exercise programme is safe and feasible in infants with single-ventricle physiology. Larger studies are needed to determine the optimal duration of passive range of motion and its effect on somatic growth.

New approach in partial cavopulmonary connection

1998 ◽  
Vol 8 (3) ◽  
pp. 364-367 ◽  
Author(s):  
Paulo. P. Paulista ◽  
Maria Virginia. T. Santana ◽  
Antonio. T. M. Henriques Neto ◽  
Valmir F. Fontes
Keyword(s):  
Limiting Factor ◽  
Late Outcome ◽  
Caval Vein ◽  
New Approach ◽  
Inferior Caval Vein ◽  
Bidirectional Glenn Shunt ◽  
Show Evidence ◽  
The Right ◽  
Cavopulmonary Connection

AbstractThe development of pulmonary atteriovenous fistulas after bidirectional cavopuomonary operat-tions, such as the bidirectional Glenn shunt and Kawashima's procedure, has raised concern. Development of these fistulas, which are more frequent than initially thought, can represnt a limiting factor in the late outcome of these patients and may even limit the indication for these of surgery. Whether the fistulas can be reversed by transforming the surgical procedures has yet to be established. In the hope of avoiding this kind of complication, thought to be caused by the lack of passage of a hypothetical hepatic factor through the pulmonary circulation. wedevelped an inverred type of gidirectional cavopulmonary connection in which the blood coming from the liver perfuses immediately both lungs. This is made possi-ble by shuntiong via an intra-atrial tunnel the blood from the superior caval vein directly to the left atrium, and the blood from the inferior caval vein to the right branch of the pulmonary trunk (keeping its bifurcation intact). We describe findings in two patients undergoing successful surgery with this technique. Serial follow -up with cohtrast echocardiography did not show evidence of arteriovenous pulmonary fistulas.Despite our numbers being small, and the time of follow-up being limited, we believe that it is importantto document these and similar cases

Long term outcome of single ventricle physiology with pulmonary restriction not undergoing Fontan repair

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
F Buendia ◽  
B Ramirez ◽  
P Gallego ◽  
J.M Oliver ◽  
S Montserrat ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Single Ventricle Physiology ◽  
Pulmonary Flow ◽  
Adult Congenital ◽  
Group 3 ◽  
Group 2

Abstract Background Patients with univentricular physiology who do not complete the palliation to Fontan are a heterogeneous group with unknown long term outcome. Aims This study aimed at describing the clinical course and long-term survival of patients with SV physiology with restricted pulmonary flow that had not undergone a Fontan type of repair. Methods From the prospectively maintained databases of the adult congenital cardiac units of five tertiary referral centers, data from all SV physiology patients were obtained. Patients completing a Fontan type palliation or developing Eisenmenger physiology and segmental pulmonary hypertension were excluded. Baseline data were recorded on the first visit at adult congenital heart disease (ACHD) unit. The primary end point was death. Results 101 patients (50.5% females) were identified. Mean age at end of follow up was 39.3±11.3 years. Of these, 45 (44.6%) were unoperated (group 1, restricted forward pulmonary flow with or without pulmonary banding), 38 (37.6%) had undergone a cavopulmonary shunt as a definitive palliation (group 2) and 18 (17.8%) had aortopulmonary shunts (group 3). The main diagnosis was double inlet left ventricle (DILV) (N: 52, 51.5%) and most of the ventricle was left (82.2%). The principal reason for not performing a Fontan repair was mean pulmonary artery pressure >18 mmHg. At initial visit at the ACHD unit patients were 32.2±11.1 years of age. 35% of the patients were in NYHA class III-IV, with no differences between groups. However, patients in group 2 had worse oxygen saturation (p=002) and higher haemoglobin (p=0.037). After a mean follow-up of 7.3±4.1 years, mortality was 20.8% (21 patients), being sudden death (7p, 6.9%) the most frequent cause. Patients in group 3 showed worse ventricular function (p=0.0001) and a trend to higher mortality that did not reach statistical significance (HR 2.7, CI 95% 0.91–8.14, P=0.07). Conclusions Patients with single ventricle physiology not undergoing Fontan repair are a population of high risk, with sudden death as main driver of mortality. Patients palliated with aortopulmonary shunts are prone to worse ventricular function and a trend to higher mortality. Funding Acknowledgement Type of funding source: Public hospital(s)

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