Tetralogy of Fallot with unilateral absent pulmonary artery

2012 ◽  
Vol 23 (3) ◽  
pp. 423-430 ◽  
Author(s):  
Murat Ugurlucan ◽  
Ahmet H. Arslan ◽  
Yahya Yildiz ◽  
Sibel Ay ◽  
Resmiye T. Besikci ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Treatment Protocol ◽  
Haemodynamic Instability ◽  
Low Oxygen ◽  
Total Correction ◽  
Mortality And Morbidity ◽  
Congenital Cardiac Malformation ◽  
Shunt Procedure ◽  
Absent Pulmonary Artery

AbstractBackgroundTetralogy of Fallot is a common congenital cardiac malformation. A rare subgroup includes unilateral absence of the pulmonary artery, either the left or the right main branch. The literature lacks an established treatment for these cases, and surgical options carry certain mortality and morbidity.Patients and methodsThere were five patients who had single pulmonary artery and received surgical treatment among the 126 patients with the diagnosis of Tetralogy of Fallot, who were admitted to our institution between July, 2010 and November, 2011. All the patients were male. Ages ranged between 12 months and 8 years. The mean body mass index was 17.1 plus or minus 3.4 kilograms per square metre. Pulmonary artery Nakata index, Nakata index Z-score, and the McGoon index were used for the quantitative assessment of the pulmonary artery and to determine the surgical strategy.ResultsUrgent modified Blalock–Taussig shunt operations were performed in two patients with very low oxygen saturation and haemodynamic instability. These patients are scheduled for corrective procedures on an elective basis. There was one patient who received an elective shunt procedure; however, the post-operative course was complicated with the overflow phenomenon and the patient underwent total correction with a check-valved patch used to close the ventricular septal defect. The patient required extracorporeal membrane oxygenator support in the post-operative period. There were two patients who underwent total correction of the pathology uneventfully. Mortality did not occur. Mean durations of hospital stay and follow-up were 14 plus or minus 13.4 days and 184.5 plus or minus 89.3 days, respectively.ConclusionOur modest series with Tetralogy of Fallot with unilateral absent pulmonary artery indicates the feasibility of surgical correction in patients with appropriate unilateral pulmonary artery size and palliative procedures when the pulmonary artery size is smaller than that predicted for the age. Multi-centre long-term data of larger series are warranted in order to establish a treatment protocol.

Tetralogy of Fallot: stent palliation or neonatal repair?

2021 ◽  
pp. 1-9
Author(s):  
Adeolu Banjoko ◽  
Golnoush Seyedzenouzi ◽  
James Ashton ◽  
Fatemeh Hedayat ◽  
Natalia N. Smith ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Surgical Repair ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Hospital Length Of Stay ◽  
Mortality And Morbidity

Abstract Surgical repair of Tetralogy of Fallot has excellent outcomes, with over 90% of patients alive at 30 years. The ideal time for surgical repair is between 3 and 11 months of age. However, the symptomatic neonate with Tetralogy of Fallot may require earlier intervention: either a palliative intervention (right ventricular outflow tract stent, ductal stent, balloon pulmonary valvuloplasty, or Blalock-Taussig shunt) followed by a surgical repair later on, or a complete surgical repair in the neonatal period. Indications for palliation include prematurity, complex anatomy, small pulmonary artery size, and comorbidities. Given that outcomes after right ventricular outflow tract stent palliation are particularly promising – there is low mortality and morbidity, and consistently increased oxygen saturations and increased pulmonary artery z-scores – it is now considered the first-line palliative option. Disadvantages of right ventricular outflow tract stenting include increased cardiopulmonary bypass time at later repair and the stent preventing pulmonary valve preservation. However, neonatal surgical repair is associated with increased short-term complications and hospital length of stay compared to staged repair. Both staged repair and primary repair appear to have similar long-term mortality and morbidity, but more evidence is needed assessing long-term outcomes for right ventricular outflow tract stent palliation patients.

scholarly journals Mortality Risk Factors in Tetralogy of Fallot Patients Undergoing Total Correction

2021 ◽  
Vol 57 (2) ◽  
pp. 151
Author(s):  
Juliana Juliana ◽  
Yan Efrata Sembiring ◽  
Mahrus Abdur Rahman ◽  
Heroe Soebroto
Keyword(s):  
Risk Factors ◽  
Developing Countries ◽  
Oxygen Saturation ◽  
Tetralogy Of Fallot ◽  
Mortality Risk ◽  
Operative Risk ◽  
Average Height ◽  
Low Oxygen ◽  
Total Correction ◽  
Ventilator Time

A total correction is a preferred treatment for Tetralogy of Fallot patients in every part of the world. However, the mortality in developing countries was as high as 6.9% to 15.3%. This was a retrospective analytic study that analyzed pre and post-operative risk factors that affected mortality on TOF patients that were performed total correction in Indonesia. A total of 47 TOF patients that were performed total correction from January 2016 to September 2019 were enrolled in this study based on the inclusion criteria. Preoperative and post-operative data were obtained from medical records. In this research, the majority of mortality was found in male patients (39.3%), while the female’s rate was lower (36.8%). Overall mortality was 38.3% and one operative death was found. The average age of patients was 84.12 months (12-210 months), whereas the average height (85.56 ± 36.17cm vs. 112.93 ± 21.73) and weight (17.22kg vs. 28.21kg) were lower for mortality patients. Some significant preoperative variables were identified as mortality risk factors such as: age below 60 months (p=0.047), smaller weight and height (p=0.008; p=0.002), abnormal hematocrit (p=0.002), and oxygen saturation below 75% (p=0.018). Significant post-operative risk factors included: temperature above 38.5⁰C (p=0.000), and ventilator time of more than 48 hours (p=0.033). In conclusion, the mortality of TOF patients undergoing a total correction in developing countries was quite high. It was associated with some risk factors, such as younger age, lower weight and height, low oxygen saturation, post-operative fever, and prolonged ventilator time.

An unusual cause of refractory persistent pulmonary hypertension of the newborn: anomalous origin of one pulmonary artery

2013 ◽  
Vol 24 (3) ◽  
pp. 543-545
Author(s):  
Nilufer Guzoglu ◽  
Fatma Nur Sari ◽  
Nahide Altug
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Innominate Artery ◽  
Persistent Pulmonary Hypertension ◽  
Anomalous Origin ◽  
Cardiac Malformation ◽  
Mortality And Morbidity ◽  
Congenital Cardiac Malformation

AbstractPersistent pulmonary hypertension of the newborn is a source of considerable mortality and morbidity. Anomalous origin of one pulmonary artery, an uncommon congenital cardiac malformation, is a rare cause of persistent pulmonary hypertension. Here, we report the case of a patient with an anomalous origin of one pulmonary artery from the innominate artery who presented with persistent pulmonary hypertension refractory to treatment.

scholarly journals Early outcome of total correction in adult tetralogy of fallot patients.

2020 ◽  
Vol 27 (06) ◽  
pp. 1304-1310
Author(s):  
Zaigham Rasool Khalid ◽  
Abdul Razzaq Mughal ◽  
Muhamamd Mujtaba Ali Siddiqui ◽  
Riaz ul Haq
Keyword(s):  
Renal Failure ◽  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Postoperative Bleeding ◽  
Case Series ◽  
Pericardial Patch ◽  
Total Correction ◽  
Female Ratio ◽  
Early Outcome

Objectives: To detect the early outcome of total correction of Tetralogy of Fallot (TOF) in adult patients of age 16 years or above. Study Design: Retrospective descriptive case series. Setting: Paediatric Cardiac Surgery Department of Faisalabad Institute of Cardiology, Faisalabad. Period: October 2016 to June 2019. Material & Methods: All consecutive patients of age 16 years or above who underwent total correction for TOF during study period were included. Surgical procedure and early outcome measures were recorded and analyzed. Results: Sixty nine patients underwent total correction for TOF during the study period. Majority of patients (n=55, 79.7%) were between16 to 25years of age. Male dominated the study population (56.5%, n=39) with male to female ratio 1.3: 1. Nine patients (13%) had coiling of MAPCA before surgery while prior palliation with Blalock Taussig shunt (BT Shunt) was seen in 5.8 % patients (n=4). All patients were operated with trans-atrial trans-pulmonary approach (n=69, 100%). Pulmonary artery was augmented with pericardial patch in 22 cases (32%) while left pulmonary artery (LPA) augmentation was done in two patients. Pulmonary valve annulus divided in 22 % of patients (n=15), RVOT was reconstructed with trans-annular pericardial patch in 10.1% patients (n=7) while Pulmonary valve was replaced with tissue valve in 7 patients (10.1%). In one patient RV to PA continuity was established with contegra. Post operatively Inotropic support was given for initial 39 ±45 hours, mean ICU stay was 39±75 hours, mean chest drainage 1086±741 ml and mean requirement for blood transfusion was 2.2 ±2.4 units. As regard early complications, six patients had tiny to small residual VSD (8.7%) while no patient developed complete heart block. Two patients had neurological damage (2.9%), one patient developed renal failure (1.45%) while chest reopening was done in two patients due to postoperative bleeding and/ or tamponade (2.9%). There was death of two patients (2.9%), one had renal failure due to low cardiac output (LCOS) and the other had stroke. Conclusion: The early outcome of complete repair of TOF in patients 16 years and above is good with a negligible mortality and limited number of complications.

Intrapulmonary artery septation for unilateral absence of the pulmonary artery

2021 ◽  
pp. 021849232110139
Author(s):  
Junichi Koizumi ◽  
Tomoyuki Iwase ◽  
Shigeto Tsuji ◽  
Takuya Goto ◽  
Kotaro Oyama ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Left Pulmonary Artery ◽  
Post Surgery ◽  
Pulmonary Artery Reconstruction ◽  
Artery Reconstruction ◽  
Absent Pulmonary Artery ◽  
Absent Left Pulmonary Artery ◽  
Tetralogy Of Fallot Repair ◽  
Blalock Taussig Shunt

We describe a seven-month-old boy with tetralogy of Fallot and an absent left pulmonary artery. Due to the diminutive size of the left pulmonary artery, we performed a native tissue left pulmonary artery reconstruction and intrapulmonary artery septation procedure with a left modified Blalock-Taussig shunt. After confirming left pulmonary artery growth, the patient underwent tetralogy of Fallot repair, removal of septation patch, and division of the Blalock-Taussig shunt. Nine months post-surgery, we confirmed his balanced lung perfusion (R/L ratio 6:4). The intrapulmonary artery septation procedure would be suitable for both the resuscitation and reconstruction of the hypoplastic absent pulmonary artery.

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