Choosing an appropriate size valve for transcatheter pulmonary valve implantation in a native right ventricle outflow tract.

Introduction: Transcatheter pulmonary valve implantation has been an effective treatment for dysfuntional right ventricular tract outflow tract (RVOT). Defining a landing zone before the intervention is crucial in patients with native RVOT. Improper sizing and undefined landing zone will lead to embolization. Methods: It is a retrospective observational study from August 2020 to December 2020 in native RVOT. Three patients who had significant Right ventricle dilatation were analyzed. The multi-slice computed tomography (MSCT) with magnetic resonance imaging and angiography data of all patients before the procedure were analyzed. All patients underwent an angiogram in the same sitting, before the procedure to assess the landing zone, valve diameter as well as the risk for coronary compression. We chose a valve based on valve area 23%-25% more than the area at the waist during balloon sizing. Results: All three patients underwent successful valve implantation. Valve sizes used were 27.5 mm in one and 32 mm in the other two. The mean RVOT gradient postprocedure was 11.5 mm Hg and pre procedure was 43 mmHg. There were no complications during the procedure or at a mean follow-up of 3.6 months. Conclusion: The balloon sizing gives the true narrowest diameter in comparison with MSCT, and increasing this area by 23%-25% will give the appropriate valve size for successful implantation.

Unanticipated complication of transcatheter correction of superior sinus venosus atrial septal defect.

Transcatheter correction of superior sinus venosus atrial septal defect (SVASD) is being considered as an alternative to surgery in selected patients. We present the case of a 42-year-old woman with SVASD and partial anomalous venous connection of the right upper pulmonary vein (RUPV), who underwent transcatheter correction with self-expanding aortic stent graft, following feasibility assessment by balloon occlusion. Hemodynamic parameters and angiography demonstrated successful closure of the SVASD without any residual shunt and unobstructed return of RUPV to the left atrium. She developed cardiac tamponade after a few hours despite pericardial drain and underwent emergency exploratory thoracotomy. This revealed leak from a small rent in the ascending aortic wall adjacent to superior vena cava (SVC) caused by barbs of the stent protruding from SVC, without any leak in SVC. This was repaired with suture and further Teflon was placed around the barbs in SVC to prevent further injury. We also discuss the possible reason for this complication, considering our successful previous two cases with the same stents. This case highlights the importance of assessing the relationship between SVC and aorta to decide about the cranial placement of the aortic stent either by computed tomography prior or by contrast aortogram during the procedure. .

Early multicenter experience of a new balloon expandable MyVal transcatheter heart valve in dysfunctional stenosed right ventricular outflow tract conduits.

BACKGROUND: Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract conduits in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has not been used for TPVI. METHODS: Patients with stenosed dysfunctional conduits from the right ventricle to pulmonary artery (RV-PA) were prestented after initial computed tomography and balloon interrogation before the implantation of MyVal. Size of MyVal was chosen based on the final diameter of the prestent. Procedural details and post-TPVI follow-up were analyzed. RESULTS: Seven patients aged 17-60 years (median 26 years) had stenosed RV-PA conduits implanted 5-17 years (median 9 years) ago for tetralogy of Fallot in three, following Ross procedure in two, repair of pulmonary stenosis, and following PA debanding in one patient each. Prestenting improved the conduit diameter from 9.3 ± 2.8 mm to 20.8 ± 1.1 mm and relieved the gradient from 87.3 ± 31.7 mmHg (50-137 mmHg) to 12.7 ± 6.4 mmHg (5-20 mmHg). A 23 mm MyVal was implanted in all the seven patients successfully; one patient needed an additional 24.5 mm MyVal valve in valve implantation for residual regurgitation. The mean fluoroscopic time and dose area product were 38.7 ± 25.3 min and 66.917 ± 39.211Gray. cm2, respectively. At a median follow-up duration of 16 months (10-22 months), all patients were asymptomatic receiving dual antiplatelet therapy with no PR and the gradient was 12.5 ± 5.8 mmHg on echocardiography. Although one patient needed an additional valve-in-valve implantation, there were no valve-related adverse events. CONCLUSIONS: Early experience of TPVI with MyVal THV in prestented conduits is encouraging with procedural success in all patients and acceptable mid-term outcomes.

Transcatheter aortic valve replacement in India-Early experience, challenges, and outcomes from a single center.

BACKGROUND: Despite the increasing popularity of transcatheter aortic valve replacement (TAVR), only about 10,000 TAVR cases have been performed in Asia to date. The procedure is still in a nascent stage in India with very few centers offering this state-of-art technique. Here, we present the early results of TAVR experience at our center. METHODS: Forty-nine patients with severe symptomatic aortic stenosis (AS) were referred to our center for TAVR from November 2015 to February 2018. Twenty-five patients underwent TAVR at our conventional cardiac catheterization laboratory under local or general anesthesia, with standby surgical team support. RESULTS: The mean age of the patients was 72.0 ± 8.1 years. The mean Society of Thoracic Surgeons score was 13.8 ± 10.2. Baseline mean ejection fraction was 50.3 ± 14.8%. Baseline mean aortic valve gradient was 55.8 ± 24.7 mmHg. There was one procedural-related death. Two of the patients required urgent surgery: one for contained annular rupture and one underwent vascular repair for femoral artery occlusion. Mild and moderate paravalvular leak was seen in 11 and 3 patients, respectively. Four patients (16%) required permanent pacemaker. Eighty percent were in New York Heart Association class I-II at discharge. One-year all-cause mortality was 8%, with no hospitalizations or major adverse cardiac event during the 1-year follow-up. CONCLUSION: Our early data clearly shows that in our country, TAVR is a good alternative for symptomatic severe AS for high surgical risk cases. Large-scale multicenter studies are required to study the real impact of TAVR in the Indian scenario. During initial years of implementation of a nationwide TAVR program, it may be prudent to focus on creating TAVR Centers of Excellence by developing an ideal hub and spokes model.

The angle of the components of the common atrioventricular valve predicts the outcome of surgical correction in patients with atrioventricular septal defect and common atrioventricular junction.

BACKGROUND: Three-dimensional echocardiography offers new insights into valvar function in atrioventricular septal defects (AVSDs). The aim of this study was to identify a morphological marker to predict the functional outcomes of left atrioventricular valves (AVVs) following the repair of AVSDs. METHODS: Twenty-nine consecutive patients were evaluated preoperatively using 2-dimensional and 3-dimensional echocardiography. The angle of the AVV relative to the crux of the heart was measured in multiplanar review mode. RESULTS: The severity of postoperative left AVV regurgitation was correlated with preoperative valvar angle, being more acute in patients with moderate or severe regurgitation (mean, 57 +/- 13 degrees vs 83 +/- 9 degrees in patients with no or mild regurgitation; P = .002). Angles < or = 59 degrees predicted severe regurgitation with 79% specificity. CONCLUSIONS: Multiplanar review of 3-dimensional data sets is valuable for the assessment of the functional morphology of AVSD valves. Using this technique, more acute AVV angles predicted increased likelihood of severe regurgitation following surgical repair.

A multiplanar three dimensional echocardiographic study of mitral valvar annular function in children with normal and regurgitant valves.

INTRODUCTION: The mitral valvar complex is difficult to visualise accurately in only two dimensions. Three-dimensional echocardiography gives new insight into the dynamic changes of intra-cardiac structures during the cardiac cycle. The aim of this study was to study the mitral annulus in systole and diastole in normal children using three-dimensional echocardiography, and to analyse the effect of regurgitation on annular function. MATERIALS AND METHODS: Three-dimensional echocardiographic datasets, acquired in 11 consecutive subjects with mitral regurgitation, and 20 normal subjects, were analysed offline using simultaneous multiplanar review. RESULTS: The mitral valvar annular area decreased in diastole, and increased in systole, in both groups. The annulus in patients with mitral regurgitation is dilated compared to normal subjects, the systolic value for those with regurgitation having a mean of 6.79 plus or minus 2.55 centimetres2/metres2, and the diastolic value a mean of 5.01 plus or minus 1.78 centimetres2/metres2, as opposed to a systolic mean value of 5.28 centimetres2/metres2 plus or minus 1.68, p = 0.091, and diastolic mean value of 3.05 centimetres2/metres2 plus or minus 0.90, in normal subjects (p less than 0.0001). The proportional change in mitral valvar annular area from systole to diastole showed a trend towards being smaller in those with mitral regurgitation, although this did not reach significance (24.8% versus 41.13%, p equal to 0.249). Analysis of subgroups of patients with moderate or severe mitral regurgitation showed mitral excursion, expressed as percentage of left ventricular length, to be significantly less than in normal subjects, at 12.78 plus or minus 5.10% versus 15.84 plus or minus 4.23% (p equal to 0.012). CONCLUSIONS: Mitral valvar annular area in children decreases in diastole, and increases in systole. In those with mitral regurgitation, the annulus is dilated and the dynamic annular function is depressed.

Hepatic changes in the failing Fontan circulation.

BACKGROUND: The failing Fontan circulation is associated with hepatic impairment. The nature of this liver injury is poorly defined. OBJECTIVE: To establish the gross and histological liver changes of patients with Fontan circulation relative to clinical, biochemical and haemodynamic findings. METHODS: Patients were retrospectively assessed for extracardiac Fontan conversion between September 2003 and June 2005, according to an established clinical protocol. Twelve patients, mean age 24.6 (range 15.8-43.4) years were identified. The mean duration since the initial Fontan procedure was 14.1 (range 6.9-26.4) years. RESULTS: Zonal enhancement of the liver (4/12) on CT was more common in patients with lower hepatic vein pressures (p = 0.007), and in those with absent cardiac cirrhosis on histological examination (p = 0.033). Gastro-oesophageal varices (4/12) were more common in patients with higher hepatic vein pressure (21 (6.3) vs 12.2 (2.2) mm Hg, p = 0.013) and associated with more advanced cirrhosis (p = 0.037). The extent of cirrhosis (7/12) was positively correlated with the hepatic vein pressure (r = 0.83, p = 0.003). A significant positive correlation was found between the Fontan duration and the degree of hepatic fibrosis (r = 0.75, p = 0.013), as well as presence of broad scars (r = 0.71, p = 0.021). Protein-losing enteropathy (5/12) occurred more frequently in patients with longer Fontan duration (11.7 (3.2) vs 17.9 (6.1) years, p = 0.038). CONCLUSIONS: Liver injury, which can be extensive in this patient group, is related to Fontan duration and hepatic vein pressures. CT scan assists non-invasive assessment. Cardiac cirrhosis with the risk of developing gastro-oesophageal varices and regenerative liver nodules, a precursor to hepatocellular carcinoma, is common in this patient group.

Correlations with operative anatomy of real time three-dimensional echocardiographic imaging of congenital aortic valvar stenosis.

OBJECTIVE: To define the anatomic characteristics of the congenitally malformed and severely stenotic aortic valve using trans-thoracic real time three-dimensional echocardiography, and to compare and contrast this with the valvar morphology as seen at surgery. DESIGN: Prospective cross-sectional observational study. SETTING: Tertiary centre for paediatric cardiology. METHODS: All patients requiring aortic valvotomy between December 2003 and July 2004 were evaluated prior to surgery with three-dimensional echocardiography. Full volume loop images were acquired using the Phillips Sonos 7500 system. A single observer analysed the images using "Q lab 4.1" software. The details were then compared with operative findings. RESULTS: We identified 8 consecutive patients, with a median age of 16 weeks, ranging from 1 day to 11 years, with median weight of 7.22 kilograms, ranging from 2.78 to 22 kilograms. The measured diameter of the valvar orifice, and the number of leaflets identified, corresponded closely with surgical assessment. The sites of fusion of the leaflets were correctly identified by the echocardiographic imaging in all cases. Fusion between the right and non-coronary leaflets was identified in half the patients. Dysplasia was observed in 3 patients, with 1 patient having nodules and 2 shown to have excrescences. At surgery, nodules were excised, and excrescences were trimmed. The dysplastic changes correlated well with operative findings, though statistically not significant. CONCLUSION: We recommend trans-thoracic real time three-dimensional echocardiography for the assessment of the congenitally malformed aortic valve, particularly to identify sites of fusion between leaflets and to measure the orificial diameter. The definition of nodularity, and the prognosis of nodules based on the mode of intervention, will need a comparative study of patients submitted to balloon dilation as well as those undergoing surgical valvotomy.

Esmolol-assisted balloon and stent angioplasty for aortic coarctation.

The objective of this study was to evaluate the effectiveness and safety of esmolol-induced negative ino- and chronotropism during stent/balloon angioplasty for aortic coarctation. Balloon angioplasty and stent placement have become widely accepted therapies for native and recurrent coarctation of the aorta (CoA). Trauma to the vessel wall and stent migration related to forward displacement of the balloon and/or stent by cardiac output, are the most common complications. Controlling stroke volume and heart rate may assist in balloon stability and accurate deployment of stents. All methods currently used to achieve this have significant limitations. We describe our experience using esmolol to control stroke volume and heart rate during balloon/stent angioplasty of CoA. We performed a retrospective review of all patients who had intravenous esmolol during percutaneous treatment of CoA. Six interventions were performed in six patients: coarctation stent angioplasty in five patients (two native coarctation) and balloon angioplasty alone in one patient. The median systolic blood pressure achieved during the procedure was 65 mmHg (range, 57-75) representing a median reduction of 40 mmHg (range, 20-80; p = 0.008) from baseline. The median heart achieved was 50 beats/min (range, 20-80), representing a median reduction of 20 beats/min (range, 15-90, p = 0.048) from baseline. Optimal stent position was obtained in all patients. Intravenous esmolol controls periprocedural hemodynamics effectively and safely during percutaneous therapy for aortic coarctation, thereby aiding accurate stent placement. Further evaluation of its use during other percutaneous left heart interventions is required.

Absent pericardium: at risk for endocarditis?

A 16-year-old girl with no previous cardiac problems presented to the casualty with septic shock. Investigations revealed endocarditis involving the mitral valve. At the time of surgery, she was noted to have a large left atrial appendage herniating into the left pleural cavity due to partial absence of the pericardium. Complete or partial absence of the pericardium is an uncommon congenital anomaly. Though various complications and presentations are reported, it has not been identified as a risk factor for endocarditis. We discuss the possible mechanism, and need for prophylaxis against endocarditis, in patients with absence of the pericardium.

Annuloplasty of the regurgitant mitral valve after myocarditis in children.

Acute myocarditis is characterized by the development of rapid life-threatening congestive heart failure and arrhythmias. In many cases with hemodynamic compromise, medical therapy and mechanical support alone are not sufficient. Various surgical procedures have been tried to bridge patients with myocarditis to both transplant and recovery. Mitral regurgitation is a frequent association with end stage cardiomyopathy and predicts poor outcome. Mitral annuloplasty is well-established in adults with ischemic and dilated cardiomyopathy and the results are superior to medical therapy alone and are comparable to cardiac transplantation. However, its effectiveness and use is not well-established in children with cardiomyopathy. We report our experience in two children.