Long-term results after palliative intra-cardiac repair for tetralogy of Fallot and diminutive pulmonary arteries

2019 ◽  
Vol 29 (8) ◽  
pp. 1036-1039
Author(s):  
Yoichi Kawahira ◽  
Kyoichi Nishigaki ◽  
Koji Kagisaki ◽  
Takuji Watanabe ◽  
Kazuki Tanimoto
Keyword(s):  
Right Ventricle ◽  
Tetralogy Of Fallot ◽  
Arterial Oxygen Saturation ◽  
Pulmonary Arteries ◽  
Systolic Pressure ◽  
Cardiac Repair ◽  
Long Term Results ◽  
Arterial Oxygen ◽  
The Right

AbstractBackground:In patients with tetralogy of Fallot with the diminutive pulmonary arteries, we sometimes have to give up the complete intra-cardiac repair due to insufficient growth of the pulmonary arteries. We have carried out palliative intra-cardiac repair using a fenestrated patch.Methods:Of all 202 patients with tetralogy of Fallot in our centre since 1996, five patients (2.5%) with the diminutive pulmonary arteries underwent palliative intra-cardiac repair using a fenestrated patch. Mean operative age was 1.8 years. Previous operation was Blalock–Taussig shunt in 4. At operation, the ventricular septal defect was closed using a fenestrated patch and the right ventricular outflow tract was enlarged. Follow-up period was 9.8 ± 2.6 years.Results:There were no operative and late deaths. Fenestration closed spontaneously on its own in four patients 2.7 ± 2.1 years after the intra-cardiac repair with a stable haemodynamics; however, the last patient with the smallest pulmonary artery index had supra-systemic pressure of the right ventricle post-operatively. The fenestration was emergently enlarged. Systemic arterial oxygen saturation was significantly and dramatically increased from 83.5 to 94% after the palliative intra-cardiac repair, and to 98% at the long term. A ratio of systolic pressure of the right ventricle to the left was significantly decreased to 0.76 ± 0.12 at the long term. Now all five patients were Ross classification class I.Conclusion:Although frequent catheter and surgical interventions were needed after the palliative intra-cardiac repair, this repair might be a choice improving quality of life with good results in patients with tetralogy of Fallot associated with the diminutive pulmonary arteries.

Balloon dilation of the right ventricular outflow tract in tetralogy of Fallot: a palliative procedure

1999 ◽  
Vol 9 (1) ◽  
pp. 11-16 ◽  
Author(s):  
Sameh M. Arab ◽  
Abdel-Fattah E. Kholeif ◽  
Salah R. Zaher ◽  
Aly M. Abdel-Mohsen ◽  
A. Samir Kassem ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Arterial Oxygen Saturation ◽  
Pulmonary Arteries ◽  
Balloon Dilation ◽  
Right Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Arterial Oxygen ◽  
Palliative Procedure ◽  
The Mean ◽  
The Right

AbstractFifteen patients requiring palliation for tetralogy of Fallot were treated by balloon dilation because of hypercyanotic spells. The mean age at dilation was 1.9 ± 0.7 years (range 0.5 – 3), and the mean weight 9.8 ± 2.1kg (range 6.0 –13.5). Dilation of the outflow tract was combined with dilation of the left and/or right pulmonary arteries in 5 patients. Successful dilation was achieved in 12 patients (80%), but failed in 3 patients with hypoplastic pulmonary arteries. In one patient, the stenosis of the right pulmonary artery could not be dilated because of a very sharp angle at the site of the stenosis. Two of the 3 patients in whom the procedure failed died of severe cyanotic spells within 24 hours of the unsuccessful procedure. No major complications occurred during or after the procedure in the cases undergoing successful dilation. The arterial oxygen saturation increased significantly, from 71 ± 5.7% to 89 ± 3.9%, immediately after the procedure (p < 0.005). During a period of follow up of 6 ± 3.7 months (range 1 – 13), the procedure was repeated on 3 occasions, and successfully accomplished in 2 of these. In conclusion, balloon dilation is a satisfactory palliative procedure for tetralogy of Fallot in those units in which total correction is not performed under 2 to 3 years of age.

Haemoptysis in a patient with tetralogy of Fallot: a combined surgical and interventional approach

2003 ◽  
Vol 13 (6) ◽  
pp. 571-573 ◽  
Author(s):  
W. Budts ◽  
P. Moons ◽  
M. Gewillig
Keyword(s):  
Right Ventricle ◽  
Tetralogy Of Fallot ◽  
Interventional Procedures ◽  
Pulmonary Arteries ◽  
Pulmonary Haemorrhage ◽  
Percutaneous Closure ◽  
Collateral Arteries ◽  
Interventional Closure ◽  
Collateral Artery ◽  
The Right

Haemoptysis may occur in patients with tetralogy of Fallot and major aorto-pulmonary collateral arteries. We describe such a patient in whom bleeding from a major aorto-pulmonary collateral artery produced severe pulmonary haemorrhage. Interventional closure of the artery could not be performed because it perfused the native pulmonary arteries. Instead, we inserted a conduit between the right ventricle and the native pulmonary arteries, followed by percutaneous closure of the collateral artery. Our patient demonstrates the increasing necessity for combined surgical and interventional procedures.

Early and late outcomes of surgical repair of double-chambered right ventricle: a single-centre experience

2020 ◽  
Vol 30 (3) ◽  
pp. 409-412
Author(s):  
Murat Surucu ◽  
İlkay Erdoğan ◽  
Birgül Varan ◽  
Murat Özkan ◽  
N. Kürşad Tokel ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Right Ventricle ◽  
Septal Defect ◽  
Surgical Intervention ◽  
Systolic Pressure ◽  
Presenting Symptoms ◽  
Long Term Follow Up ◽  
The Right

AbstractObjective:Double-chambered right ventricle is characterised by division of the outlet portion of the right ventricle by hypertrophy of the septoparietal trabeculations into two parts. We aim to report our experiences regarding the presenting symptoms of double-chambered right ventricle, long-term prognosis, including the recurrence rate and incidence of arrhythmias after surgery.Methods:We retrospectively investigated 89 consecutive patients who were diagnosed to have double-chambered right ventricle and underwent a surgical intervention from 1995 to 2016. The data obtained by echocardiography, cardiac catheterisation, and surgical findings as well as post-operative follow-up, surgical approaches, post-operative morbidity, mortality, and cardiac events were evaluated.Results:Median age at the time of diagnosis was 2 months and mean age at the time of operation was 5.3 years. Concomitant cardiac anomalies were as follows: perimembranous ventricular septal defect (78 patients), atrial septal defect (9 patients), discrete subaortic membrane (32 patients), right aortic arch (3 patients), aortic valve prolapse and/or mild aortic regurgitation (14 patients), and left superior caval vein (2 patients). The mean follow-up period was 4.86 ± 4.6 years. In these patients, mean systolic pressure gradient in the right ventricle by echocardiography before, immediately, and long-term after surgical intervention was 66.3, 11.8, and 10.4 mmHg, respectively. There were no deaths during the long-term follow-up period. Surgical reinterventions were performed for residual ventricular septal defect (2), residual pulmonary stenosis (1), and severe tricuspid insufficiency (1).Conclusion:The surgical outcomes and prognosis of double-chambered right ventricle are favourable, recurrence and fatal arrhythmias are unlikely in long-term follow-up.

Repair of tetralogy of Fallot associated with atrioventricular septal defect

1998 ◽  
Vol 8 (1) ◽  
pp. 105-112 ◽  
Author(s):  
Tomás̆ Tláskal ◽  
Bohumil Huc̆ín ◽  
Martin Kostelka ◽  
Václav Chaloupecký ◽  
Jan Marek ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Pulmonary Stenosis ◽  
Pulmonary Arteries ◽  
Pulmonary Regurgitation ◽  
Atrioventricular Septal Defect ◽  
Arterial Stenosis ◽  
Long Term Results

AbstractTetralogy of Fallot, when associated with atrioventricular septal defect permitting shunting at ventricular level, represents a complex cyanotic congenital malformation. Experience with surgical repair is limited, and results vary considerably. Between 1984 and 1996, we repaired 14 consecutive patients with this combination seen in our center. Their ages ranged from 8 months to 21 years (median 7.4 years). Six (42.9%) had Down's syndrome. In eight patients the correct diagnosis was made using echocardiogra phy alone. In the remaining six patients, who had previously-constructed arterial shunts and/or suspected pulmonary arterial stenosis, catheterization and angiocardiography were also performed. The repair consisted of double patch closure of the septal defect, reconstruction of two atrioventricular orifices, and relief of pulmonary stenosis at all levels. In five patients with a hypoplastic pulmonary trunk, a monocusp transannular patch (four patients) or an allograft (one patient) was used for restroration of continuity from the right ventricle to the pulmonary arteries. Patch enlargement of one or both pulmonary arteries was necessary in five patients. One patient (7.1%) died early, and another late. The twelve surviving (85.8%) patients have been followed for 1.2–12.5 years after surgery (median 4.9 years, mean 5.9 ± 3.9 years). During the follow-up, reoperation was necessary for repair of residual ventricular septal defect and pulmonary regurgitation in two patients, and closure of an atrial septal defect and alteration to left atrioventricular valvar regurgitation in one patient. Seven patients are in class I of the New York Heart Association, four in class II, and one in class III. Tetralogy of Fallot associated with atrioventricular septal defect can be corrected with low mortality and good long-term results. Residual lesions, however, have a tendency to progress, especially when seen in combination. After surgery, all patients need long-term close follow-up.

scholarly journals Successful surgery for a right ventricle rhabdomyoma in a neonate

2020 ◽  
Vol 48 (4) ◽  
pp. 280-284
Author(s):  
M. V. Tarayan ◽  
E. S. Efremov ◽  
I. O. Bondareva ◽  
N. V. Shkarina ◽  
S. N. Kavaidin
Keyword(s):  
Blood Flow ◽  
Right Ventricle ◽  
Postoperative Period ◽  
Emergency Surgery ◽  
Tumor Resection ◽  
Systolic Pressure ◽  
Cardiac Tumors ◽  
Advanced Tumor ◽  
The Right

Primary cardiac tumors are very rare in children. Rhabdomyoma is the most common benign tumor in fetuses and neonates. Most cases do not require any surgical intervention due to absence of clinical symptoms and a high rate of spontaneous regression within the first year of life. However, some neonates can have significant abnormalities of intracardial hemodynamics related to the obstruction of the left and right ventricle outflows; this is an indication to emergency surgery. As a rule, complete or partial resection of the tumor mass provides uneventful postoperative course and is not associated with a relapse of rhabdomyoma in the long-term.We present a clinical case of a 15-days old neonate who underwent an emergency surgery due to advanced tumor obstruction of the blood flow in the right ventricle outflow tract (RVOT). At preoperative echocardiography, there was a pulmonary artery systolic pressure gradient of 90 mm Hg. Moderate hypoxemia (SaO2 90%), breathing rate of up to 55 per minute, together with echocardiographic results, indicated the impaired pulmonary blood flow and the need for the tumor resection. The tumor was completely resected through the right ventricle access with cardiopulmonary bypass and cardioplegia, with subsequent autologous pericardium patching of the right ventricle. The postoperative period was uneventful; the patient was extubated at day 2 and discharged at day 11 after surgery. The diagnosis of rhabdomyoma was confirmed histologically. At one month after surgery, no additional tumor masses were found in the heart chambers and septum.Rhabdomyoma causing severe obstruction of the RVOT in a newborn is a rare life-threatening complication of the natural course of benign heart tumors in children. The presence of dyspnea at rest, moderate desaturation, and echocardiographic data determined the rejection of the conservative management commonly adopted in most cases of cardiac rhabdomyomas, and were indications for an emergency surgery. In the long-term postoperative period, magnetic resonance imaging should be done to exclude tuberous sclerosis.

Bubble contrast echocardiography in detecting pulmonary arteriovenous malformations after modified Fontan operations

2001 ◽  
Vol 11 (5) ◽  
pp. 505-511 ◽  
Author(s):  
Eva Strömvall Larsson ◽  
Laszlo Solymar ◽  
Bengt O. Eriksson ◽  
Anne de Wahl Granelli ◽  
Mats Mellander
Keyword(s):  
Arteriovenous Malformations ◽  
Contrast Echocardiography ◽  
Arterial Oxygen Saturation ◽  
Pulmonary Arteries ◽  
Positive Contrast ◽  
Pulmonary Angiography ◽  
Arterial Oxygen ◽  
Colloid Solution ◽  
Pulmonary Arteriovenous Malformations ◽  
The Right

The development of pulmonary arteriovenous malformations is a well-known complication after Fontan operations, and may result in significant morbidity due to increasing arterial desaturation. We compared the use of bubble contrast echocardiography and pulmonary angiography in detecting such malformations. We also examined which anatomical and haemodynamic variables were associated with their development. Our study includes 20 patients who had undergone modified Fontan procedures, 10 with atriopulmonary and 10 with total cavopulmonary connections, in Gothenburg between 1980 and 1991. All patients underwent cardiac catheterisation and pulmonary angiography. Bubble contrast echocardiography was performed at the same time, with injection of agitated polygelin colloid solution (Haemaccel, Hoechst) into the right and left pulmonary arteries, respectively. Transoesophageal echocardiography was used to detect the appearance of bubble contrast in the pulmonary venous atrium. The aim was also to evaluate the role of hepatic venous blood. Of the 20 patients, 9 (45%) had a positive contrast echocardiography study, compared with only 2 (10%) detected by pulmonary angiography. Patients with positive contrast echocardiography had a significantly lower arterial oxygen saturation than those with negative studies, both at rest (88% vs 95%, p < 0.01) and during exercise testing (78% vs 89%, p = 0.01). Bubble contrast echocardiography is much more sensitive in detecting pulmonary arteriovenous malformations than pulmonary angiography. By injecting echo contrast into the right and left pulmonary arteries, the method can be made highly selective. Pulmonary arteriovenous malformations develop much more frequently in patients with the Fontan circulation than previously reported.

Total correction of tetralogy of Fallot without “routine”preoperative cardiac catheterization—management of 99 patients

1994 ◽  
Vol 4 (3) ◽  
pp. 262-266
Author(s):  
Ayse Sarioglu ◽  
Gülhis Batmaz ◽  
Mehmet Salih Bilal ◽  
Irfan Levent Saltik ◽  
Gül Saylam ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Thoracic Aorta ◽  
Cardiac Catheterization ◽  
Pulmonary Arteries ◽  
Systolic Pressure ◽  
Left Ventricular ◽  
Descending Thoracic Aorta ◽  
Total Correction ◽  
Echocardiographic Examination ◽  
The Right

SummaryBetween January 1989 and March 1993, total correction was performed in 99 patients with tetralogy of Fallot without submitting them to prior cardiac catheterization. The age of the patients ranged from 1.33 to 18 years (mean 5.33±3.77). After complete echocardiographic examination, the diameters of the right and left pulmonary arteries at the prebranching point and the descending thoracic aorta at the diaphragm were measured by cross-sectional echocardiography and the McGoon ratio was calculated. Total correction was performed in all patients with a McGoon ratio greater than 1.7. In none of the patients were the sizes of the pulmonary artery measured by echocardiography smaller than the measurements obtained during surgery. Transannular patching was performed in 76 patients. A conduit from the right ventricle to the pulmonary arteries was constructed in two patients with coronary arterial anomalies. Postrepair right ventricular to left ventricular systolic pressure ratios were between 0.25 and 0.85 (mean 0.54±0.13). There were two hospital deaths, neither being related to the diagnostic method used nor the criteria for surgery. We conclude that the diagnosis of tetralogy of Fallot together with measurements of pulmonary arteries and descending thoracic aorta can safely and reliably be achieved echocardiographically. The McGoon ratio can be adapted to echocardiography and total correction can be performed successfully based on echocardiographic examination.

Efficacy and safety of balloon dilation as palliative treatment for tetralogy of Fallot

1994 ◽  
Vol 4 (3) ◽  
pp. 255-261 ◽  
Author(s):  
Gurleen K. Sharland ◽  
Shakeel A. Qureshi ◽  
Edmund J. Ladusans ◽  
Jonathon M. Parsons ◽  
Edward J. Baker ◽  
...  
Keyword(s):  
Oxygen Saturation ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Arterial Oxygen Saturation ◽  
Balloon Dilation ◽  
Right Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Arterial Oxygen ◽  
The Mean ◽  
The Right

AbstractTwenty-six patients requiring palliation for the tetralogy of Fallot were treated by balloon dilation. The mean age at dilation was 8.5 months (range 0.5−20.6) and the mean weight 6.7 kg (range 2.6−10.7). Successful dilation was achieved in 25 patients. The mean ratio of the balloon to the diameter of the pulmonary outflow tract was 1.6 (range 1.2−2.0). The aortic oxygen saturation increased from mean±SD of 80±12.4% before to 91±4.3% after dilation (p<0.05). The pulmonary arterial oxygen saturation rose from 64±10.7% to 80±7.7% (p<0.05). Transient hypotension and arterial desaturation were noted in 19 patients during inflation of the balloon. One patient had a cardiac arrest prior to dilation and died afterwards. Seven patients had cyanotic spells related to the procedure. Attempts to cross the pulmonary valve resulted in infundibular perforation by the guide wire in one patient. Of the 24 survivors who initially had successful dilation procedures, three have needed a Blalock-Taussig shunt. Palliation without a shunt was achieved in the remaining 21 patients. In 12 patients, repeat angiography, 2.5−20 months (mean 9.1) after dilation, showed an increase in the diameter of the outflow tract from 7.1±2.1 mm before to 9.5±2.0 mm after dilation (p<0.05). Diameters of the pulmonary arteries increased from 6.3±2.2 mm on the right and 6.8±1.9 mm on the left before to 9.2±2.2 mm and 9.3±2.5 mm respectively after dilation (p<0.05 for both branches). Surgical correction has been achieved in 22 patients, 21 of these 2−22 months after balloon dilation and the other patient 20 months after construction of a shunt. At operation, the leaflets of the pulmonary valve were noted to be torn in five patients. In 12 patients, there was extensive scarring of the infundibulum. Balloon dilation of the right ventricular outflow tract achieves adequate palliation and may result in an increase in the diameter of the pulmonary valve and branches of the pulmonary trunk. It should be considered as an alternative to shunt procedures in children with tetralogy of Fallot.

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