Tetralogy of Fallot with coarctation of the aorta

1994 ◽  
Vol 4 (1) ◽  
pp. 75-78 ◽  
Author(s):  
Masaaki Yoshigi ◽  
Kazuo Momma ◽  
Yasuharu Imai

SummaryWe report a two-year-old boy with a rare combination of tetralogy of Fallot and aortic coarctation. The obstruction in this patient had an hourglass-like configuration, with a pressure difference of 15 mm Hg. There was a right aortic arch, bilateral brachiocephalic arteries, and persistent left superior caval vein. Percutaneous transluminal balloon angioplasty was performed to lower the resistance to the left ventricular ejection, considered a risk factor for subsequent corrective surgery of tetralogy. Six months later, he underwent combined repair of tetralogy of Fallot and coarctectomy with end-to-end anastomosis, thus avoiding the future potential for restenosis. During surgery, a ligament was found extending from the right subclavian artery to the distal side of the coarctation. The perioperative course was uneventful, indicating the success of the preoperative balloon angioplasty. We speculate that the embryogenesis of the coarctation could be related to an abnormal involution of the aortic arches.

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Despina Toader ◽  
Alina Paraschiv ◽  
Petrișor Tudorașcu ◽  
Diana Tudorașcu ◽  
Constantin Bataiosu ◽  
...  

Abstract Background Left ventricular noncompaction is a rare cardiomyopathy characterized by a thin, compacted epicardial layer and a noncompacted endocardial layer, with trabeculations and recesses that communicate with the left ventricular cavity. In the advanced stage of the disease, the classical triad of heart failure, ventricular arrhythmia, and systemic embolization is common. Segments involved are the apex and mid inferior and lateral walls. The right ventricular apex may be affected as well. Case presentation A 29-year-old Caucasian male was hospitalized with dyspnea and fatigue at minimal exertion during the last months before admission. He also described a history of edema of the legs and abdominal pain in the last weeks. Physical examination revealed dyspnea, pulmonary rales, cardiomegaly, hepatomegaly, and splenomegaly. Electrocardiography showed sinus rhythm with nonspecific repolarization changes. Twenty-four-hour Holter monitoring identified ventricular tachycardia episodes with right bundle branch block morphology. Transthoracic echocardiography at admission revealed dilated left ventricle with trabeculations located predominantly at the apex but also in the apical and mid portion of lateral and inferior wall; end-systolic ratio of noncompacted to compacted layers > 2; moderate mitral regurgitation; and reduced left ventricular ejection fraction. Between apical trabeculations, multiple thrombi were found. The right ventricle had normal morphology and function. Speckle-tracking echocardiography also revealed systolic left ventricle dysfunction and solid body rotation. Abdominal echocardiography showed hepatomegaly and splenomegaly. Abdominal computed tomography was suggestive for hepatic and renal infarctions. Laboratory tests revealed high levels of N-terminal pro-brain natriuretic peptide and liver enzymes. Cardiac magnetic resonance evaluation at 1 month after discharge confirmed the diagnosis. The patient received anticoagulants, antiarrhythmics, and heart failure treatment. After 2 months, before device implantation, he presented clinical improvement, and echocardiographic evaluation did not detect thrombi in the left ventricle. Coronary angiography was within normal range. A cardioverter defibrillator was implanted for prevention of sudden cardiac death. Conclusions Left ventricular noncompaction is rare cardiomyopathy, but it should always be considered as a possible diagnosis in a patient hospitalized with heart failure, ventricular arrhythmias, and systemic embolic events. Echocardiography and cardiac magnetic resonance are essential imaging tools for diagnosis and follow-up.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A E Vijiiac ◽  
D Muraru ◽  
F Jarjour ◽  
K Kupczynska ◽  
C Palermo ◽  
...  

Abstract Background The right atrium (RA) is a highly dynamic chamber with 3 mechanical functions (reservoir, conduit, booster pump) and prognostic implications in heart failure (HF) and pulmonary hypertension (PH). However, RA function and its interplay with the right ventricular (RV) performance in patients (pts) with reduced left ventricular ejection fraction (LVEF) and without PH remain to be clarified. Methods We used three-dimensional echocardiography to study 55 pts (61 ± 14 years, 43 men) with LVEF < 40% no more than mild tricuspid regurgitation (TR), and maximum velocity of the TR jet < 3 m/s. We measured the three-dimensional RA total, passive, active ejection volumes (EV) and the respective emptying fractions (EF). In addition, we compared RV volumes and ejection fraction (RVEF) between patients with normal and abnormal RA function. Results Mean LVEF was 30 ± 7%. Mean echo-derived pulmonary vascular resistance was 1.64 ± 0.54 Wood units. 28 pts (51%) had reduced RA reservoir function (total EF = 34 ± 9%), 34 pts (62%) had reduced RA conduit function (passive EF = 15 ± 4%), and 10 pts (18%) had reduced RA pump function (active EF = 11 ± 3%). Pts with reduced RA reservoir function showed larger RV end-systolic volume (RVESV 124 ± 48ml vs. 90 ± 32ml; p = 0.004) and lower RVEF (38 ± 8% vs. 46 ± 6%; p < 0.001) than pts with normal RA function. Pts with reduced RA conduit function showed smaller RV stroke volume (RVSV 65 ± 19 ml vs. 80 ± 22ml; p = 0.009). Pts with impaired RA pump function showed larger RVESV (142 ± 45ml vs. 99 ± 41ml; p = 0.02) and lower RVEF (36 ± 6% vs. 43 ± 8%; p = 0.006). RVESV was positively correlated with total (r2 = 0.47, p < 0.001), passive (r2 = 0.29, p = 0.03) and active (r2 = 0.39, p = 0.003) RAEV, while it was negatively correlated with total (r2=-0.41, p = 0.002), passive (r2=-0.34, p = 0.01) and active (r2=-0.31, p = 0.02) RAEF. RVSV showed a positive correlation with both total (r2 = 0.4, p = 0.002) and passive (r2 = 0.41, p = 0.002) RAEV. Finally, RVEF was positively correlated with total (r2 = 0.51, p < 0.001), passive (r2 = 0.47, p < 0.001), and active (r2 = 0.36, p = 0.007) RAEF. Conclusions RA dysfunction is not uncommon in pts with reduced LVEF, even in the absence of PH. In these pts, RA function is associated with significant changes in RV function. The RA acts as a dynamic modulator of RV pump function by redistributing RV filling and ejection force among reservoir, conduit and pump functions in the setting of altered hemodynamics. The clinical and prognostic significance of RA function in pts with reduced LVEF warrant further studies.


1962 ◽  
Vol 203 (6) ◽  
pp. 1141-1144 ◽  
Author(s):  
Jay M. Levy ◽  
Emmanuel Mesel ◽  
Abraham M. Rudolph

Simultaneous right and left ventricular stroke volumes were measured with electromagnetic flow probes in open-chest, anesthetized dogs. Atrial ectopic beats with normal ventricular depolarization produced differences between right and left ventricular stroke output, although the right and left ventricular pressures were proportionately reduced to an equal extent. This imbalance in volume ejected was a result of the differences in diastolic level, related to peak systolic pressure, in the aorta compared with pulmonary artery. With ventricular ectopic beats, the stimulated ventricle failed to develop the same percentage of control pressure as did the contralateral ventricle. The difference between aortic and pulmonary flow was thus less marked with right ventricular ectopic beats, and exaggerated with left ventricular ectopic beats.


2019 ◽  
Vol 21 (8) ◽  
pp. 906-913 ◽  
Author(s):  
Imran Rashid ◽  
Adil Mahmood ◽  
Tevfik F Ismail ◽  
Shamus O’Meagher ◽  
Shelby Kutty ◽  
...  

Abstract Aims The optimal timing for pulmonary valve replacement in asymptomatic patients with repaired Tetralogy of Fallot (rTOF) and pulmonary regurgitation remains uncertain but is often guided by increases in right ventricular (RV) end-diastolic volume. As cardiopulmonary exercise testing (CPET) performance is a strong prognostic indicator, we assessed which cardiovascular magnetic resonance (CMR) parameters correlate with reductions in exercise capacity to potentially improve identification of high-risk patients. Methods and results In all, 163 patients with rTOF (mean age 24.5 ± 10.2 years) who had previously undergone CMR and standardized CPET protocols were included. The indexed right and left ventricular end-diastolic volumes (RVEDVi, LVEDVi), right and left ventricular ejection fractions (RVEF, LVEF), indexed RV stroke volume (RVSVi), and pulmonary regurgitant fraction (PRF) were quantified by CMR and correlated with CPET-determined peak oxygen consumption (VO2) or peak work. On univariable analysis, there was no significant correlation between RVEDVi and PRF with peak VO2 or peak work (% Jones-predicted). In contrast, RVEF and RVSVi had significant correlations with both peak VO2 and peak work that remained significant on multivariable analysis. For a previously established prognostic peak VO2 threshold of <27 mL/kg/min, receiver-operating characteristic curve analysis demonstrated a Harrell’s c of 0.70 for RVEF (95% confidence interval 0.61–0.79) with a sensitivity of 88% for RVEF <40%. Conclusion In rTOF, CMR indices of RV systolic function are better predictors of CPET performance than RV size. An RVEF <40% may be useful to identify prognostically significant reductions in exercise capacity in patients with varying degrees of RV dilatation.


1970 ◽  
Vol 26 (1) ◽  
pp. 26-31
Author(s):  
F Rahman ◽  
S Banerjee ◽  
CM Ahmed ◽  
MS Uddin ◽  
Khirul Anam ◽  
...  

This prospective ongoing study conducted in University Cardiac Center, BSMMU, Dhaka from July 2004 to April 2006. 100 patients (mean age 52.4±6.2 years) underwent Percutaneous Transluminal Coronary angioplasty and stenting (PTCA & stenting) were evaluated. This study was designed to evaluate the short term angiographic and clinical results of stentangioplasty during hospital stay. The study group of 100 patients consisted of 88 (88%) men and 12 (12%) women. About risk factors 36 (36%) had hypertension, 30 (30%) were smoker, 20 (20%) suffered from diabetes mellitus, 14 (14%) had family history of ischaemic heart disease. Average Left ventricular ejection fraction was 54.2±7. Target vessel PTCA were done on 130 vessels, intracoronary stent implanted in 124 vessels, direct stenting was done in 80 cases, failed PTCA were in 4 (4%) cases, and three patients had dissection. The native vessels had a mean reference diameter of 2.89 mm and their luminal diameter increased significantly after percutaneous coronary intervention (PCI). Thombolysis in myocardial infarction (TIMI) flow analysis showed most of the patients had TIMI-1 flow (95,73%) before the procedure and maximum patients achieved TIMI-3 flow (91, 70%) after the procedure with significant clinical improvement. All the patients were discharged by one to three days of the procedure with improvement of their clinical condition. So PTCA and Stenting is a safe and effective technique with high procedural success rate and good short-term (hospital) clinical results in the native coronary artery lesions. Key words: Coronary artery diseases; PTCA and stenting. DOI: 10.3329/jbcps.v26i1.4230 J Bangladesh Coll Phys Surg 2008; 26: 26-31


1995 ◽  
Vol 5 (2) ◽  
pp. 155-160 ◽  
Author(s):  
Sandra Giusti ◽  
Adele Borghi ◽  
Sofia Redaelli ◽  
Philipp Bonhoeffer ◽  
Isabella Spadoni ◽  
...  

SummaryBalloon dilation of the aortic valve was performed in 20 consecutive neonates with critical aortic stenosis using an approach achieved by cutting down on the right carotid artery. The age of the patients ranged from one to 25 days (mean seven days) and their weight from 2.1 to 4.0 kg (mean 3.16 kg). All patients were evaluated before cardiac catheterization with cross-sectional and Doppler echocardiography so as to keep the catheterization procedure as short as possible. Balloon dilation was accomplished in all patients. The only complication was an apical perforation by the guide wire in two cases. The ensuing pericardial effusion was immediately drained with pericardiocentesis and the subsequent course of the procedure was uneventful. Immediate results showed dramatic improvement in cardiovascular conditions. The transvalvar pressure gradient fell from 80±40 to 27±20 mm Hg (p<0.001). Left ventricular ejection fraction evaluated by echocardiography increased from 30±21% before dilation to 54±18% 24-48 hours after the procedure (p<0.001). In all patients, the procedure was free from vascular complications. Aortic regurgitation was documented after the procedure in 11 patients, being severe in one, moderate in five and trivial in five. Seven patients died, although in only one was the death related directly to the procedure itself. Six patients died because of associated lesions despite an immediate satisfactory result of the balloon valvoplasty. The 13 surviving patients are doing well, and are receiving no medications. During a mean follow-up of 25 months (range 2-54 months), four patients have developed restenosis. One underwent surgical valvotomy at one year of age. The second was successfully redilated through the same approach at two months of age. The other two have a significant gradient, as assessed by Doppler measurements (60 and 70 mm Hg), with normal systolic ventricular function. Two patients have moderate aortic regurgitation. Balloon dilation achieved through cutdown on the right carotid artery is a safe and effective alternative to surgery in neonates with isolated aortic stenosis. The unfavorable results are mainly due to associated anomalies.


2014 ◽  
Vol 5 (4) ◽  
pp. 110-117
Author(s):  
Sergey Pavlovich Marchenko ◽  
Gennadiy Grigorevich Khubulava ◽  
Alexey Borisovich Naumov ◽  
Anastasia Alexeevna Seliverstova ◽  
Natalia Dmitrievna Cipurdeeva ◽  
...  

The diagnosis and treatment of patients with hemodynamic disorders is basing of the understanding the physiology and pathophysiology of cardiovascular system. The relationship between length of muscle fibers and power reduction were first time revealed by Fick. Otto Frank was formulated fundamental principles of contractility of cardiomyocytes. Straub and Wiggers in 1914 was described the mail principles of the right ventricle work. Ernest Starling was performed a series of experiments, which explore a dependence of left ventricular ejection from venous inflow and elastic resistance of the aorta. In 1914 Ernest Starling was published research result, which describe how the mechanical energy of heart beats is depend from length fiber. Another essential part of knowledge of this problem was discovered by Aurtur Guyton experiments. Guyton has been established that there is a linear relationship between the pressure in the right atrium and the venous return. The lack of quantitative assessment of volume status has led to a qualitative approach, de-scribed by the term “response to the volume load.” However, as pointed out by some authors [16, 29], it is important to remember that the response to the preload is not a pathological condition. A quantitative approach to the assessment of volume status is based on the concept of Guyton on average system pressure filling, theoretically independent of cardiac function. This approach is used clinically. In this review article we describe possibility of clinical application of all knowledge of this questions.


2016 ◽  
Vol 27 (5) ◽  
pp. 890-894 ◽  
Author(s):  
David A. Briston ◽  
Aarthi Sabanayagam ◽  
Ali N. Zaidi

AbstractObesity is increasingly prevalent, and abnormal body mass index is a risk factor for cardiovascular disease. There are limited data published regarding body mass index and CHD. We tested the hypothesis that body mass index and obesity prevalence are increasing in patients with tetralogy of Fallot over time by analysing time since surgery, age, height, weight, and body mass index among tetralogy of Fallot patients and demographic data from age-matched controls. NYHA class and left ventricular ejection fraction were analysed in adults. Body mass index was categorised into normal, overweight, and obese in this single-centre, retrospective chart review. Data were collected from 137 tetralogy of Fallot patients (71 men:66 women), of whom 40 had body mass index >25 kg/m2. Tetralogy of Fallot patients aged <6 years had lower body mass index (15.9 versus 17.1; p=0.042) until 16–20 years of age (27.4 versus 25.4; p=0.43). For adult tetralogy of Fallot patients, the mean body mass index was 26.5 but not statistically significantly different from the control cohort. Obese adult patients had significantly higher average NYHA class compared with those of normal weight (p=0.03), but no differences in left ventricular ejection fraction by echocardiography (p=0.55) or cardiac MRI (p=0.26) were noted. Lower body mass index was observed initially in tetralogy of Fallot patients, but by late adolescence no significant difference was observed. As adults, tetralogy of Fallot patients with higher body mass index had increased NYHA class but similar left ventricular ejection fraction.


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