scholarly journals Technique Using a Titanium Plate to Reconstruct the Inferior Sternal Cleft in Pentalogy of Cantrell: Lessons From Four Patients

Author(s):  
Linyun Xi ◽  
Chun Wu ◽  
Zhengxia Pan ◽  
Ming Xang

Abstract BackgroundBy reviewing the outcomes of four patients, we summarize our experience with the strategy of using a titanium plate to reconstruct the inferior sternal cleft in pentalogy of Cantrell (POC).MethodsThis was a retrospective analysis of 4 patients who visited our department between January 2000 and June 2020 concurrent with POC. All four patients underwent an operation, as well as cardiac ultrasound and thoracoabdominal computed tomography (CT). Cardiac malformations achieved satisfactory correction according to echocardiographs. A titanium plate was used to repair the sternal and supraumbilical abdominal defects. The hospital course, operative data, and outpatient records were reviewed.ResultsAll 4 patients had partial sternal clefts, and 4 patients underwent a single-stage operation. All 4 cases of ectopia cordis were eventually repositioned. The defect in the sternum and supraumbilical abdomen was repaired using a titanium plate. One patient with double-outlet right ventricle (DORV) developed low cardiac output syndrome and pulmonary infection, and symptomatic treatment was administered until discharge. The mean follow-up time ranged from 9 months to 10 years. No patient developed pectus excavatum, and there were no cases of retrosternal seroma or pneumothorax. The titanium plate was migratory in the second patient and was dislodged at another hospital 3.5 years postoperation, and a fibreboard was formed in the area where sternal cleft; the beating heart could not be observed outside the thoracoabdominal or thoracic wall. In the first patient, the titanium plate was torn with a small fissure at 2 years postoperation, but the fissure was not enlarged during follow-up. In the other two patients, the appearance of both the abdominal wall and lower sternum and cardiac function were good. The New York Heart Association function class was I in all four patients.ConclusionThe use of a titanium plate to construct the neosternum can yield a satisfactory exterior appearance of the thorax with a partial sternal cleft, but long-term outcomes need to be examined further.

2020 ◽  
Author(s):  
Qiang Ji ◽  
YuLin Wang ◽  
Ye Yang ◽  
Hao Lai ◽  
WenJun Ding ◽  
...  

Abstract Background: Septal myectomy has been a standard treatment option for patients with hypertrophic obstructive cardiomyopathy (HOCM) and drug refractory symptoms. However, there are only a few experienced myectomy centers in the world so far, mainly because of high technical difficulty of myectomy. From our clinical experience, the use of the mini-invasive surgical instruments during myectomy may be beneficial to reduce the technical difficulty. This study reports the preliminary experience regarding transaortic septal myectomy using mini-invasive surgical instruments for the treatment of patients with HOCM and drug refractory symptoms, and evaluates the early results following myectomy.Methods Between March 2016 and March 2019, consecutive HOCM patients were included in this analysis who underwent isolated transaortic septal myectomy using the mini-invasive surgical instruments. Intraoperative, in-hospital and follow-up results were analyzed.Results A total of 168 eligible patients (83 males, mean 56.8 ± 12.3 years) were included. Midventricular obstruction was recorded in 7 (4.2%) patients. All included patients underwent transaortic septal myectomy with a mean aortic cross-clamping time of 36.0 ± 8.1 minutes. Nine (5.4%) patients received repeat aortic cross-clamping during surgery. Surgical mortality was 0.6%. Five (3.0%) patients developed complete atrioventricular block and required permanent pacemaker implantation. The median follow-up time was 6 months. No follow-up deaths occurred with a significant improvement in New York Heart Association functional status. The maximum gradients decreased sharply from the preoperative value (11.6 ± 7.4 mmHg vs. 94.4 ± 2 2.6 mmHg, p<0.001). The median degree of mitral regurgitation fell to 1.0 (vs. 3.0 preoperatively, p<0.001) with a significant reduction in the proportion of moderate or more regurgitation (1.2% vs. 57.7%, p<0.001).Conclusions The use of the mini-invasive surgical instruments may be beneficial to reduce the technical difficulty of transaortic septal myectomy procedure. Transaortic septal myectomy using the mini-invasive surgical instruments may be associated with favorable results.


Author(s):  
Peter Kubuš ◽  
Jana Rubáčková Popelová ◽  
Jan Kovanda ◽  
Kamil Sedláček ◽  
Jan Janoušek

Background Cardiac resynchronization therapy (CRT) is rarely used in patients with congenital heart disease, and reported follow‐up is short. We sought to evaluate long‐term impact of CRT in a single‐center cohort of patients with congenital heart disease. Methods and Results Thirty‐two consecutive patients with structural congenital heart disease (N=30) or congenital atrioventricular block (N=2), aged median of 12.9 years at CRT with pacing capability device implantation, were followed up for a median of 8.7 years. CRT response was defined as an increase in systemic ventricular ejection fraction or fractional area of change by >10 units and improved or unchanged New York Heart Association class. Freedom from cardiovascular death, heart failure hospitalization, or new transplant listing was 92.6% and 83.2% at 5 and 10 years, respectively. Freedom from CRT complications, leading to surgical system revision (elective generator replacement excluded) or therapy termination, was 82.7% and 72.2% at 5 and 10 years, respectively. The overall probability of an uneventful therapy continuation was 76.3% and 58.8% at 5 and 10 years, respectively. There was a significant increase in ejection fraction/fractional area of change ( P <0.001) mainly attributable to patients with systemic left ventricle ( P =0.002) and decrease in systemic ventricular end‐diastolic dimensions ( P <0.05) after CRT. New York Heart Association functional class improved from a median 2.0 to 1.25 ( P <0.001). Long‐term CRT response was present in 54.8% of patients at last follow‐up and was more frequent in systemic left ventricle ( P <0.001). Conclusions CRT in patients with congenital heart disease was associated with acceptable survival and long‐term response in ≈50% of patients. Probability of an uneventful CRT continuation was modest.


Circulation ◽  
2000 ◽  
Vol 102 (suppl_3) ◽  
Author(s):  
Jian Xin Qin ◽  
Takahiro Shiota ◽  
Patrick M. McCarthy ◽  
Michael S. Firstenberg ◽  
Neil L. Greenberg ◽  
...  

Background —Infarct exclusion (IE) surgery, a technique of left ventricular (LV) reconstruction for dyskinetic or akinetic LV segments in patients with ischemic cardiomyopathy, requires accurate volume quantification to determine the impact of surgery due to complicated geometric changes. Methods and Results —Thirty patients who underwent IE (mean age 61±8 years, 73% men) had epicardial real-time 3-dimensional echocardiographic (RT3DE) studies performed before and after IE. RT3DE follow-up was performed transthoracically 42±67 days after surgery in 22 patients. Repeated measures ANOVA was used to compare the values before and after IE surgery and at follow-up. Significant decreases in LV end-diastolic (EDVI) and end-systolic (ESVI) volume indices were apparent immediately after IE and in follow-up (EDVI 99±40, 67±26, and 71±31 mL/m 2 , respectively; ESVI 72±37, 40±21, and 42±22 mL/m 2 , respectively; P <0.05). LV ejection fraction increased significantly and remained higher (0.29±0.11, 0.43±0.13, and 0.42±0.09, respectively, P <0.05). Forward stroke volume in 16 patients with preoperative mitral regurgitation significantly improved after IE and in follow-up (22±12, 53±24, and 58±21 mL, respectively, P <0.005). New York Heart Association functional class at an average 285±144 days of clinical follow-up significantly improved from 3.0±0.8 to 1.8±0.8 ( P <0.0001). Smaller end-diastolic and end-systolic volumes measured with RT3DE immediately after IE were closely related to improvement in New York Heart Association functional class at clinical follow-up (Spearman’s ρ=0.58 and 0.60, respectively). Conclusions —RT3DE can be used to quantitatively assess changes in LV volume and function after complicated LV reconstruction. Decreased LV volume and increased ejection fraction imply a reduction in LV wall stress after IE surgery and are predictive of symptomatic improvement.


2014 ◽  
Vol 8 ◽  
pp. CMC.S14016 ◽  
Author(s):  
Carlo Lombardi ◽  
Valentina Carubelli ◽  
Valentina Lazzarini ◽  
Enrico Vizzardi ◽  
Filippo Quinzani ◽  
...  

Amino acids (AAs) availability is reduced in patients with heart failure (HF) leading to abnormalities in cardiac and skeletal muscle metabolism, and eventually to a reduction in functional capacity and quality of life. In this study, we investigate the effects of oral supplementation with essential and semi-essential AAs for three months in patients with stable chronic HF. The primary endpoints were the effects of AA's supplementation on exercise tolerance (evaluated by cardiopulmonary stress test and six minutes walking test (6MWT)), whether the secondary endpoints were change in quality of life (evaluated by Minnesota Living with Heart Failure Questionnaire—MLHFQJ and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels. We enrolled 13 patients with chronic stable HF on optimal therapy, symptomatic in New York Heart Association (NYHA) class II/III, with an ejection fraction (EF) <45%. The mean age was 59 ± 14 years, and 11 (84.6%) patients were male. After three months, peak VO2 (baseline 14.8 ± 3.9 mL/minute/kg vs follow-up 16.8 ± 5.1 mL/minute/kg; P = 0.008) and VO2 at anaerobic threshold improved significantly (baseline 9.0 ± 3.8 mL/minute/kg vs follow-up 12.4 ± 3.9 mL/minute/kg; P = 0.002), as the 6MWT distance (baseline 439.1 ± 64.3 m vs follow-up 474.2 ± 89.0 m; P = 0.006). However, the quality of life did not change significantly (baseline 21 ± 14 vs follow-up 25 ± 13; P = 0.321). A non-significant trend in the reduction of NT-proBNP levels was observed (baseline 1502 ± 1900 ng/L vs follow-up 1040 ± 1345 ng/L; P = 0.052). AAs treatment resulted safe and was well tolerated by all patients. In our study, AAs supplementation in patients with chronic HF improved exercise tolerance but did not change quality of life.


2019 ◽  
Vol 56 (2) ◽  
pp. 385-392
Author(s):  
Kevin M Veen ◽  
Mostafa M Mokhles ◽  
Jolien W Roos-Hesselink ◽  
Bas R Rebel ◽  
Johanna J M Takkenberg ◽  
...  

AbstractOBJECTIVESSince 1988, our centre employs vertical plication repair with deattachment and reattachment of the tricuspid valve for Ebstein anomaly. This study describes the characteristics and long-term outcomes of our single-centre cohort.METHODSData from all patients operated on between 1988 and 2016 were retrospectively collected. Kaplan–Meier analyses were done for survival data and mixed models were used to analyse longitudinally collected clinical and echocardiography data.RESULTSThirty-six patients (mean age: 25.4 ± 15.9 years, 36% male) were operated on using the Carpentier–Chauvaud 21 (58%) or Cone repair 15 (42%). One patient (3%) died in hospital. Two late deaths were observed, yielding a survival of 97 ± 3% at 25 years. Reoperation was performed in 6 patients after a mean follow-up of 14.1 ± 10.3 years, resulting in a freedom of reoperation of 80 ± 8% at 25 years. During follow-up, predicted probability of being in New York Heart Association III/IV did not exceed 10%. Modelling longitudinal evolution of tricuspid regurgitation showed no major changes over time. Additionally, a rigid ring repair was associated with a higher probability of tricuspid regurgitation, especially after the first years after the operation. A full Cone repair was associated with less progression of tricuspid regurgitation over time.CONCLUSIONSRepair of Ebstein abnomaly is associated with low mortality and morbidity, acceptable reoperation rate and excellent valve function over time, especially in patients with completed Cone repair. Therefore, we conclude that in our centre, repair of Ebstein abnomaly is a durable technique to treat patients.


2016 ◽  
Vol 43 (5) ◽  
pp. 392-396 ◽  
Author(s):  
Gianluca Lucchese ◽  
Lucia Rossetti ◽  
Giuseppe Faggian ◽  
Giovanni B. Luciani

Temporary tricuspid valve detachment improves the operative view of certain congenital ventricular septal defects (VSDs), but its long-term effects on tricuspid valve function are still debated.From 2002 through 2012, we performed a prospective study of 68 children (mean age, 1.28 ± 1.01 yr) who underwent transatrial closure of VSDs following temporary tricuspid valve detachment. Sixty patients had conoventricular and 8 had mid-muscular VSDs. All were in sinus rhythm. Seventeen patients had systemic pulmonary artery pressures. Preoperative echocardiograms showed trivial-to-mild tricuspid regurgitation in 62 patients and tricuspid dysplasia with severe regurgitation in 6 patients. Patients were clinically and echocardiographically monitored at 30 postoperative days, 3 months, 6 months, every 6 months thereafter for the first 2 years, and then once a year.No in-hospital or late death was observed at the median follow-up evaluation of 5.9 years. Mean intensive care unit and hospital stays were 1.6 ± 1.1 and 7.3 ± 2.7 days, respectively. Residual small VSDs occurred in 3 patients, and temporary atrioventricular block in one. After VSD repair, 62 patients (91%) had trivial or mild tricuspid regurgitation, and 6 moderate. Five of these last had severe tricuspid regurgitation preoperatively and had undergone additional tricuspid valve repair during the procedure. The grade of residual tricuspid regurgitation remained stable postoperatively, and no tricuspid stenosis was documented. All patients were in New York Heart Association class I at follow-up.Temporary tricuspid valve detachment is a simple and useful method for a complete visualization of certain VSDs without incurring substantial tricuspid dysfunction.


2020 ◽  
Author(s):  
Xiufang Kong ◽  
Lili Ma ◽  
Peng Lv ◽  
Xiaomeng Cui ◽  
Rongyi Chen ◽  
...  

Abstract Background: Takayasu arteritis (TA) is a large-vessel vasculitis that can involve pulmonary arteries (PAs). We studied multiple clinical characteristics related to pulmonary artery involvement (PAI) in TA patients.Methods: We enrolled 216 patients with TA from a large prospective cohort. PAI was assessed in each patient based on data from magnetic resonance angiography/computed tomography angiography. Pulmonary hypertension, cardiac function, and pulmonary parenchymal abnormalities were evaluated further in patients with PAI based on echocardiography, New York Heart Association Functional Classification and pulmonary computed tomography, respectively. These abnormalities related to PAI were followed up to evaluate treatment effects.Results: PAI was detected in 56/216 (25.93%) patients, which involved the pulmonary trunk, main PAs and small vessels in the lungs. Among patients with PAI, 28 (50%) patients were accompanied by pulmonary hypertension, which was graded as ‘severe’ in 9 (16.07%), ‘moderate’ in 10 (17.86%) and mild in 9 (16.07%). Forty (71.43%) patients had cardiac insufficiency (IV: 6, 10.71%; III: 20, 35.71%; II: 14, 25.00%). Furthermore, 21 (37.50%) patients presented with abnormal parenchymal features in the area corresponding to PAI (e.g., the mosaic sign, infarction, bronchiectasis). During follow-up, two patients died due to abrupt pulmonary thrombosis. In the remaining patients, the abnormalities mentioned above improved partially after routine treatment.Conclusions: PAI is very common in TA patients. PAI can cause pulmonary hypertension, cardiac insufficiency and pulmonary parenchymal lesions, which worsen the prognosis.


2018 ◽  
Vol 5 (3) ◽  
pp. 252-258 ◽  
Author(s):  
Morten Kvistholm Jensen ◽  
Lothar Faber ◽  
Max Liebregts ◽  
Jaroslav Januska ◽  
Jan Krejci ◽  
...  

Abstract Aims We analysed the impact of bundle branch block (BBB) and pacemaker (PM) implantation on symptoms and survival after alcohol septal ablation (ASA) in patients with hypertrophic cardiomyopathy (HCM). Methods and results Among 1416 HCM patients from the Euro-ASA registry, 58 (4%) patients had a PM and 64 (5%) patients had an implantable cardioverter-defibrillator (ICD) before ASA. At latest follow-up (5.0 ± 4.0 years) after ASA, 118 (8%) patients had an ICD and 229 (16%) patients had a PM. In patients without an implantable device prior to ASA 13% had a PM and 5% had an ICD implanted following ASA. New onset BBB was present in 44% (right BBB in 31%) of patients without previous BBB. At latest follow-up, we found no associations between BBB and New York Heart Association (NYHA) Class 3–4 [odds ratio (OR) 0.98, 95% confidence interval (CI) 0.63–1.51; P = 0.91] or Canadian Cardiovascular Society (CCS) Class 3–4 (OR 1.5, CI 0.32–6.7; P = 0.62), respectively, and no associations between PM and NYHA Class 3–4 (OR 1.2, CI 0.70–2.0; P = 0.52) or CCS 3–4 (OR 1.3, CI 0.24–6.6; P = 0.79), respectively. The survival after ASA was not reduced in patients with BBB [hazard ratio (HR) 0.73, CI 0.53–1.01; P = 0.06] or PM (HR 0.78, CI 0.52–1.17; P = 0.24). Conclusions Development of BBB or need for a PM after ASA in patients with obstructive HCM was not associated with inferior symptomatic outcome or reduced survival, thus concerns for the negative impact of impaired cardiac conduction on the clinical outcome after ASA were not confirmed.


Author(s):  
Uberto Da Col ◽  
Simone Perticoni ◽  
Enrico Ramoni

Objective Although effective, Carpentier technique for mitral regurgitation presents two “Achille's heel”: the resection of the whole prolapsing section of posterior mitral leaflet (PML) including chordae tendinae and the annular distortion due to plication. An alternative technique of limited PML resection, which preserves mitral anatomy decreasing the impact on valve function, and 9-year outcome are presented. Methods Since April 2005 till March 2014, of 205 patients affected by mitral prolapse scheduled for repair (mitral valve repair), 54 patients have been included in the study. The rationale of the new technique was to limit PML resection to achieve a fair reduction of the prolapsing scallop(s) height, to avoid leaflet and annular distortion, and to spare the coaptation surface and other substantial structures. According to the observation that the posterior smooth zone of PML is quite free from chordal insertions, an elliptical slice of tissue was resected from this area. Annuloplasty and neochordal insertion when indicated completed the procedure. Results Up to 9 years of follow-up was 98% complete. One inhospital death, two late noncardiac deaths, one redo operation due to endocarditis were reported. On late follow-up, 92% patients were on New York Heart Association class I. Late echocardiography showed stability of repair (regurgitation grade of ≤1 in 92% of patients). Nearly two third of valves preserved good PML mobility. Conclusions The parannular elliptical posterior leaflet resection, providing excellent stable midterm results, seems to be a safe alternative method for repair of PML prolapse. It avoids distortion and weakening of annulus and leaflet, and it allows restoring a proper coaptation surface and maintains a satisfactory PML motion.


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