A rare association of the absence of left pulmonary artery with ventricular septal defect, pulmonary hypertension, and more interestingly, patent ductus arteriosus in an adult patient: Case report and literature review.

Key Clinical Message: In this study, we introduced one of the rarest concomitants of the absence of left pulmonary artery (LPA), which was seen in our patient along with patent ductus arteriosus (PDA) and ventricular septal defect (VSD). Abstract: Unilateral absence of pulmonary artery (UAPA) is a congenital heart disease in association with other abnormalities such as tetralogy of Fallot and septal defects or isolated in 30% of cases and occurs in the right lung in two thirds of cases. Our case is a 33-year-old man who was hospitalized with symptoms of cough, shortness of breath, and hemoptysis. The echocardiography revealed a large ventricular septal defect, absent left pulmonary artery, and severe pulmonary hypertension (PH) along with patent ductus arteriosus. These findings were confirmed by CT angiography. This association has rarely been found in past studies. Due to PH and pulmonary infection, the patient was treated with intravenous prostaglandin and antibiotics. However, in cases of timely diagnosis and treatment of UAPA, fatal complications such as pulmonary hypertension, morbidity, and mortality are reduced. This case emphasizes the importance of awareness of this abnormality and its associated anomalies to enable early diagnosis and treatment.

Aortic dilation in adults with repaired tetralogy of Fallot: a single-centre study.

BACKGROUND: Tetralogy of Fallot is the most prevalent cyanotic CHD. With the advent of advanced surgical methods, the majority of tetralogy of Fallot patients reach adulthood. However, many need re-intervention for the residual anomalies including residual right ventricular outflow obstruction, pulmonary regurgitation, residual ventricular septal defects, and progressive aortic dilatation. Aortic dilation could lead to aortic regurgitation or dissection requiring surgical correction. In the current study, we aimed to determine the prevalence and outcomes of aortic root dilatation in adults with repaired tetralogy of Fallot in our tertiary care centre. METHODS: In this retrospective study, 730 consecutive patients with history of repaired tetralogy of Fallot were included. Aortic diameter at the level of annulus, the sinus of Valsalva, sinotubular junction, and the ascending aorta as measured by echocardiography were evaluated. Prevalence of outcomes necessitating re-intervention including aortic regurgitation and dissection were recorded. RESULTS: The mean size of annulus, sinus of Valsalva, sinotubular-junction, and ascending aorta in the latest available echocardiography of patients were 2.4+/-0.4 cm, 3.3+/-0.5 cm, 2.9+/-0.5cm, and 3.2+/-0.5cm, respectively. Prevalence of dilatation of sinus of Valsalva, dilation of Ascending aorta, sinotubular-junction, and aortic annulus was 28.7%, 21%, 8.3%, and 1 %, respectively. Five patients had severe aortic regurgitation (0.6%) and underwent surgical repair. One of these patients presented with acute aortic dissection. CONCLUSION: Aortic dilation is common in tetralogy of Fallot but prevalence of redo surgery for aortic dilation, regurgitation, and adverse events including acute dissection is low.

Short-term outcomes following total correction of tetralogy of fallot in adult patients.

OBJECTIVES: Tetralogy of Fallot (TOF) is a common congenital heart disease which should be corrected. The recommended time for the Tetralogy of Fallot Total Correction (TFTC) surgery is during the infancy for the possible difficulties during the surgery and the related issues. However, sometimes TOF is diagnosed and managed during the adulthood. METHODS: This study is a descriptive and retrospective one which included all patients who underwent TFTC at the age of 15-year and older in 10 years (between the years 2010 and 2020) to identify short-term (in-hospital mortality, ICU stay, postoperative bleeding, respiratory complications after the surgery such as pulmonary edema, pneumonia, etc.) and one-year (left ventricle ejection fraction (LVEF), right ventricle (RV) ejection fraction, the severity of tricuspid and aortic regurgitation after surgery) outcomes. All data were taken from medical records at Rajaie Cardiovascular Medical and Research Center. Data were analyzed using SPSS 22. RESULTS: 94 patients with the mean ± SD age of 26.7 ± 9.6 years were enrolled. Most of them were male (59.6%) (P-value: 0.009). In-hospital mortality in our study were 5.3%. Tricuspid regurgitation (TR) was significantly resolved after the surgery (P-value: 0.006). Of 17 (18.1%) patients with small or hypoplastic pulmonary artery (PA) branches, 14 patients had acceptable PA branch size after surgery. CONCLUSION: TFTC at an older age is safe with acceptable results. Age is not a contraindication for TFTC and surgery should be recommended if the patients are diagnosed with TOF in adulthood. Also, the TOF diagnosis should be considered in adult patients with suspicious signs and symptoms.

Midterm prognosis following total correction of tetralogy of fallot in adult patients.

Background: Tetralogy of Fallot is a common congenital heart disease characterized by cyanosis. The primary treatment approach involves corrective surgery typically performed within the first year of life to achieve complete resolution. However, certain patients may undergo surgery at an older age. This study seeks to assess the efficacy of surgery by examining the midterm outcomes of total correction of Tetralogy of Fallot when performed in older individuals. Methods: This interventional-longitudinal study focused on patients who underwent complete surgery to correct tetralogy of Fallot at an advanced age of over 15 years. All of the participants were referred to the Shahid Rajaei Heart and Vascular Center, which is a referral center for congenital heart diseases in Iran, between 2010 and 2020. The surgical procedures for these patients involved primary total correction of tetralogy of Fallot or surgery following by shunt implantation. Prior to the surgery, the necessary information was gathered from the patients' medical records. The patients were then monitored over a 5-year period, during which they received regular check-ups from cardiologist with fellowship in adult congenital heart disease. Results: A total of 94 participants were enrolled in the study, with an average age of 26.7 ± 9.6 years. Notably, the majority of the participants were male. The study reported a late mortality rate of 3.2%. Furthermore, 17 patients, constituting 18% of the cohort, underwent a secondary surgical procedure. This secondary surgery encompassed 14 cases of Pulmonary Valve Replacement (14.8%) and 3 cases of Ventricular Septal Defect repair (3.1%). Conclusion: While the optimal age for total correction of Tetralogy of Fallot is conventionally considered to be within the first year of life, this study demonstrated that surgical intervention performed at a later stage of life can yield favorable midterm prognoses. It is imperative to emphasize that individuals unable to undergo surgery at the ideal age due to a multitude of factors should not be deprived of the potential benefits associated with surgical intervention.

Right ventricular echocardiographic remodeling after pulmonary valve replacement in repaired Tetralogy of Fallot.

BACKGROUND: Many patients with repaired tetralogy of Fallot require reoperation in the medium to long-term for residual pulmonary valve regurgitation. Best timing for pulmonary valve replacement remains controversial. A balance needs to be found between protecting the patients from permanent right ventricular damage and insertion of a prosthetic valve with its inherent issues. In the current study we sought to investigate the right and left ventricular functional recovery following valve replacement in our tertiary care institution. RESULTS: In a retrospective cross-sectional study patients with history of total correction of tetralogy of Fallot who had undergone pulmonary valve replacement due to severe pulmonary regurgitation between 2003-2018 were evaluated for post intervention right and left ventricular functional recovery. Clinical and full echocardiographic data before and after the surgery were recorded and compared. There was statistically significant improvement in RV size and function post pulmonary valve replacement. There was no statistically significant improvement in left ventricular systolic function. Twenty percent of patient had persisting severe right ventricular enlargement at least twelve months post-surgery. No patient had fully normalized right ventricular size and function in follow-up. CONCLUSIONS: Pulmonary valve replacement leads to improvement in right ventricular size and function in patients with repaired tetralogy of Fallot. However normalization of functional parameters did not occur and the majority of the patients have residual right and left ventricular dysfunction following redo valve replacement surgery.

A giant aneurysm of sinus of Valsalva presenting with frequent syncopal attacks.

Sinus of Valsalva aneurysm is a rare congenital or acquired aortic root anomaly. The aneurysm has the potential to compress the adjacent structures or rupture to other cardiac chambers with fatal consequences. Patients might be asymptomatic or present with nonspecific cardiac symptoms. Once the diagnosis is made, prompt surgical repair is generally required. Here, we present a young woman with frequent syncope who was found to have a giant sinus of Valsalva aneurysm as the underlying etiology.

Prevalence of Structural Heart Diseases Detected by Handheld Echocardiographic Device in School-Age Children in Iran: The SHED LIGHT Study.

Background: Structural heart disease (SHD) has great impacts on healthcare systems, creating further public health concerns. Proper data are scant regarding the magnitude of the affected population by SHD. Objectives: This study aimed to determine the prevalence of SHD among children and adolescents in an Iranian population. Methods: In this population-based study, a multistage cluster-random sampling was used to choose schools from the Tehran urban area. All students were examined using a handheld Vscan device by echocardiographer, and the results were concurrently supervised and interpreted by cardiologists. All the major findings were reevaluated in hospital clinics. Results: Of 15,130 students (6-18 years, 52.2% boys) who were examined, the prevalence of individuals with congenital heart disease (CHD) and cardiomyopathy was 152 (10.046 per 1,000 persons) and 9 (0.595 per 1,000 persons), respectively. The prevalence of definite and borderline rheumatic heart disease (RHD) was 30 (2 per 1,000 persons) and 113 (7.5 per 1,000 persons), correspondingly. Non-rheumatic valvular heart disease (VHD) was also detected in 465 (30.7 per 1,000 persons) students. Of all the pathologies, only 39 (25.6%) cases with CHD and 1 (0.007%) cases with RHD had already been diagnosed. Parental consanguinity was the strongest predictor of CHD and SHD (odds ratio [OR]: 1.907, 95% CI, 1.358 to 2.680; P < 0.001 and OR, 1.855, 95% CI, 1.334 to 2.579; P < 0.001, respectively). The female sex (OR, 1.262, 95% CI, 1.013 to 1.573; P = 0.038) and fathers' low literacy (OR, 1.872, 95% CI, 1.068 to 3.281; P = 0.029) were the strongest predictors of non-rheumatic VHD and RHD, correspondingly. Conclusions: The implementation of echocardiographic examinations for detecting SHD among young population is feasible which detected SHD prevalence in our population comparable to previous reports. Further studies are required to delineate its economic aspects for community-based screening.

Cardiac remodeling after atrial septal defects device closure.

BACKGROUND: Trans-catheter device closure of secundum type atrial septal defects (ASDs) has now become the treatment of choice. Device closure affects the hemodynamics and function of the heart. In the current study, we investigated the remodeling of the heart as assessed by advanced echocardiographic parameters following successful device closure. METHODS: A prospective cohort study was conducted and 45 patients who were eligible for ASD device closure were evaluated for volumetric, functional, Doppler, strain, and strain rate data of left and right atrium and ventricle pre-procedural and 48 h post-procedure RESULTS: In a prospective cohort study 45 patients who were eligible for ASD device closure; atrial and ventricular volumetric, functional, Doppler, strain, and strain rate data were recorded and compared pre-procedurally and 48 h post-procedure.We found significant changes in the left ventricular (LV) end diastolic volume index (p-value = .03), right ventricular (RV) diameter (p-value = <.001), left atrial (LA) volume index (p-value = .05), right atrial (RA) volume index (p-value = .001), and right and left sided E/e' ratio with a p-value of .001 and .004, respectively. Our findings showed a significant reduction in the strains of the right and left atria and the right ventricular free wall after ASD device closure. LV global longitudinal strain decreased after the procedure but did not reach statistical significance. CONCLUSION: ASD patients have impaired global strains compared to normal defined ranges. LA, RA, and RV strains show significant reduction after device closure. Decline in LA function following closure was greater in those with larger ASDs. In adult patients undergoing the procedure, abnormal LA function is a clinically relevant issue demanding pre and post- procedural precautions and treatment.

Multiple congenital visceral abnormalities as a rare cause of pulmonary arterial hypertension.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare, progressive disorder. PAH is caused by a wide spectrum of pathologies but the cause remains undetermined on many occasions and patients are classified in the idiopathic group. CASE PRESENTATION: Here we report a young woman with rare congenital visceral abnormalities presenting with severe pulmonary hypertension. CONCLUSIONS: Pulmonary hypertension is a complex disorder. Search for uncommon conditions that lead to pulmonary hypertension is necessary to determine the best management options.

Safety and feasibility of simultaneous left and right heart catheterization via single-arm arterial and venous access.

BACKGROUND: Catheterization via the radial artery has become the method of choice for evaluation of the coronary arteries; however in patients requiring simultaneous coronary and right heart catheterization upper extremity access is not commonly used. AIMS: In the present study we aimed to assess whether simultaneous left and right heart catheterization via the radial artery and antecubital vein might increase the vascular access site complications. METHODS: In this prospective cohort study, 49 patients with congenital and valvular heart disease requiring both left and right heart catheterization were enrolled, and rates of vascular access complications, including radial artery obstruction (RAO), were compared to 49 subjects in the control group who underwent catheterization only via radial artery access. RESULTS: No major vascular complications occurred in the study population. Post-procedural radial artery obstruction was detected in 14 patients (28.5%) in the venous-radial artery group and 7 (14.2%) in the control group (P = 0.09). Age, sex, body mass index, wrist circumference, hemoglobin concentrations, and history of smoking, diabetes mellitus, hypertension, and anticoagulant use did not affect the rate of radial pulse perseverance. However, shorter times of radial compression device removal were significantly related to lower rates of radial artery obstruction in both groups. CONCLUSION: Right and left heart catheterization via an upper-extremity route in patients with congenital and valvular heart disease is safe and practical with greater patient comfort and no serious vascular complications. A longer duration of radial compression is an important predictor of RAO, regardless of using additional upper-extremity venous access.

Balloon-Expandable Cheatham-Platinum Stents Versus Self-Expandable Nitinol Stents in Coarctation of Aorta: A Randomized Controlled Trial.

OBJECTIVES: This study sought to compare the safety and efficacy of the balloon-expandable stent (BES) and the self-expandable stent (SES) in the endovascular treatment of coarctation of aorta. BACKGROUND: Coarctoplasty with stents has conferred promising results. Although several nonrandomized studies have approved the safety and efficacy of the BES and the SES, no high-quality evidence exists for this comparison. METHODS: In the present open-label, parallel-group, blinded endpoint randomized pilot clinical trial, adult patients with de novo native aortic coarctation were randomized into Cheatham-platinum BES and uncovered nitinol SES groups. The primary outcome of the study was a composite of procedural and vascular complications. The secondary outcomes of the study consisted of the incidence of aortic recoarctation, thoracic aortic aneurysm/pseudoaneurysm formation, and residual hypertension at a 12-month follow-up. RESULTS: Among 105 patients who were screened between January 2017 and December 2019, 92 eligible patients (32 women [34.8%]) with a median age of 30 years (IQR: 20-36 years) were randomized equally into the BES and SES groups. The composite of procedural and vascular complications occurred in 10.9% of the BES group and 2.2% of the SES group (odds ratio: 0.18; 95% CI: 0.02-1.62; P = 0.20). Aortic recoarctation occurred in 5 patients (5.4%), 3 patients (6.5%) in the BES group and 2 patients (4.3%) in the SES group (odds ratio: 0.65; 95% CI: 0.10-4.09; P = 0.64). Only 1 patient (1.1%) was complicated by aortic pseudoaneurysm. Hypertension control was achieved in 50% of the study population, with an equal distribution in the 2 study groups at the 12-month follow-up. CONCLUSIONS: Both the BES and the SES were safe and effective in the treatment of native coarctation.

Incidental detection of ruptured sinus of Valsalva aneurysm and coronary abnormality in a patient with penetrating chest trauma.

Aortic sinus of Valsalva aneurysm is a rare congenital or acquired anomaly with a potential for rupture into adjacent cardiac chambers. Ruptured aneurysm of the sinus of Valsalva leads to volume overload of the receiving chamber and should be repaired as soon as the diagnosis is made. Here, we present a young patient with history of chest trauma leading to discovery of an asymptomatic ruptured sinus of Valsalva aneurysm.

Fast vs. ultraslow thrombolytic infusion regimens in patients with obstructive mechanical prosthetic valve thrombosis: a pilot randomized clinical trial.

AIMS: Thrombolysis is an alternative to surgery for mechanical prosthetic valve thrombosis (MPVT). Randomized clinical trials have yet to test the safety and efficacy of a proposed ultraslow thrombolytic infusion regimen. METHODS AND RESULTS: This single-centre, open-label, pilot randomized clinical trial randomized adult patients with acute obstructive MPVT to an ultraslow thrombolytic regimen [25 mg of recombinant tissue-type plasminogen activator (rtPA) infused in 25 h] and a fast thrombolytic regimen (50 mg of rtPA infused in 6 h). If thrombolysis failed, a repeated dose of 25 mg of rtPA for 6 h was administered in both groups up to a cumulative dose of 150 mg or the occurrence of a complication. The primary outcome was a complete MPVT resolution (>75% fall in the obstructive gradient by transthoracic echocardiography, <10° limitation in opening and closing valve motion angles by fluoroscopy, and symptom improvement). The key safety outcome was a Bleeding Academic Research Consortium type III or V major bleeding. Overall, 120 patients, including 63 (52.5%) women, at a mean age of 36.3 ± 15.3 years, were randomized. Complete thrombolysis success was achieved in 51 patients (85.0%) in the ultraslow-regimen group and 47 patients (78.3%) in the fast-regimen group [odds ratio 1.58; 95% confidence interval (CI) 0.25-1.63; P = 0.34]. One case of transient ischaemic attack and three cases of intracranial haemorrhage (absolute risk difference -6.6%; 95%CI -12% -0.3%; P = 0.07) were observed only in the fast-regimen group. CONCLUSION: The ultraslow thrombolytic regimen conferred a high thrombosis resolution rate without major complications. Such findings should be replicated in more adequately powered trials.

Case Report of a 33-year-old with Ebstein malformation of tricuspid valve and hypertrophic cardiomyopathy.

Ebstein malformation of tricuspid valve is a congenital disease of tricuspid valve with associated right ventricular cardiomyopathy. Hypertrophic cardiomyopathy is a form of inherited left ventricular cardiomyopathy caused by sarcomeric protein gene mutations with inherent risks of sudden cardiac death. Here we report a rare case with co-occurrence of Ebstein malformation of tricuspid valve and hypertrophic cardiomyopathy in a young patient.

Transhepatic device closure of large atrial septal defect.

Transcatheter closure of secundum-type atrial septal defects has become the treatment of choice in the majority of cases. Femoral venous access is the standard rout for device implantation. Anatomic abnormalities of venous system including interrupted inferior caval vein with azygous continuation can make the percutaneous procedure more complicated. In such instances, alternative methods of transjugular or transhepatic approach or surgical repair should be considered. We present the case of a 50-year-old male with secundum-type atrial septal defect and a rare form of segmental interruption of inferior caval vein and describe successful atrial septal defect closure through transfemoral approach.

An adult case with absence of left pulmonary artery and partial anomalous pulmonary venous connection: Case report and literature review.

Unilateral absence of pulmonary artery is a rare congenital disorder that can remain asymptomatic until adulthood. Absence of left pulmonary artery (ALPA) has been reported in one-third of these patients. We are the first to introduce an adult case of ALPA associated with partial anomalous pulmonary venous connection.

Paradigm Shift in the Management of Isolated Interrupted Aortic Arch in Adulthood.

An interrupted aortic arch (IAA) is a rare type of congenital heart disease, and few patients survived into the adulthood. Surgical reconstruction is still the recommended approach, despite its complexity and considerable complication. In addition, patients with IAA usually suffer from several other important congenital heart anomalies, which increase the complexity of surgical management of IAA. Although endovascular therapy has replaced surgery in the treatment of the majority of non-IAA, its applicability in IAA is still matter of debate. In the present review, we have discussed about various therapeutic solutions of IAA, and present a stepwise approach for its endovascular management.