Benefits of Balloon‐Dilatable Bilateral Pulmonary Artery Banding in Patients With Hypoplastic Left Heart Syndrome and Other Complex Cardiac Anomalies

Objectives

The purpose of this study was to evaluate the potential of balloon‐dilatable bilateral pulmonary artery banding (b‐PAB) and its impact on the configuration of the pulmonary artery (PA).

Background

We have previously used balloon‐dilatable b‐PAB as first‐stage palliation for patients with hypoplastic left heart syndrome (HLHS) and other complex cardiac anomalies.

Methods

Two pliable tapes were placed around each branch of the PA and tightened with 7–0 polypropylene sutures in a manner that allowed for the subsequent adjustment of PA diameters. We retrospectively examined the adjustability of PA diameters by balloon dilation and the need for surgical PA angioplasty at later stages.

Results

From January 2010 to October 2013, we performed b‐PAB in 8 patients, including 3 borderline cases between biventricular repair (BVR) and univentricular repair (UVR). The b‐PAB procedures were performed at a median age of 6.5 days (range, 2–10 days). Balloon dilations were performed in 10 lesions in 4 patients. All of the procedures were performed safely. Two patients reached definite BVR. The remaining 6 patients underwent open palliative procedures with univentricular physiologies that resulted in 2 deaths unrelated to the initial b‐PAB. In all but 1 of the patients, the PA configuration was properly maintained and did not require surgical pulmonary angioplasty.

Conclusions

Balloon‐dilatable b‐PAB can be performed safely and prevents PA distortion at later stages. This technique should be considered for patients with complex cardiac anomalies if uncertainty exists regarding the optimal surgical strategy (BVR or UVR) in early infancy. (J Interven Cardiol 2015;28:594–599)

     

A better approach for left ventricular training in transposition of the great arteries and intact interventricular septum: Bidirectional cavopulmonary anastomosis and pulmonary artery banding

Objective: Management of the patients with transposition of the great arteries and intact ventricular septum may be challenging beyond the newborn period. Herein, we would like to present our alternative strategy for training the left ventricle in these patients.
Methods: Six patients with transposition of the great arteries and intact ventricular septum were evaluated in our clinic. Two of them were palliated with Glenn procedure and pulmonary banding as a definitive treatment strategy at other centers. Four patients were operated on and a bidirectional cavopulmonary anastomosis in combination with pulmonary artery banding was performed (stage‐1: palliation and ventricular training) in our center. In four out of these six patients, arterial switch operation was performed with takedown and direct re‐anastomosis of the superior vena cava to right atrium after an interstage period of 21‐30 months (stage‐2: anatomical repair).
Results: Any mortality was not encountered. The left ventricular mass indices increased from 18‐32 to 44‐74 g/m2 in patients undergoing the anatomical repair. All of the patients were uneventfully discharged following the second stage. The mean follow‐up period was 20 months (9‐32 months) following stage 2. All of the patients are doing well with trivial neoaortic regurgitation and normal biventricular function.
Conclusions: Bidirectional cavopulmonary anastomosis with pulmonary artery banding may be a promising left ventricle training approach in ventriculoarterial discordance when compared to the traditional pulmonary artery banding with concomitant systemic‐to‐pulmonary artery shunt procedures which still carry a significant interstage morbidity and mortality.     

Development of a device for transcatheter pulmonary artery banding: evaluation in animals.

AIMS: Pulmonary artery banding (PAB) is the first palliation in infants with complex congenital heart disease and elevated pulmonary blood flow. In older patients with corrected transposition of the great arteries, it may be used to re-train the left ventricle. To date, the only option is surgical. We report the development and the evaluation of a device for transcatheter PAB. METHODS AND RESULTS: We intended to implant a pulmonary artery (PA) reducer percutaneously between the native pulmonic valve and the pulmonary bifurcation. Immediately following its insertion, we planned to implant a balloon expandable stent inside the restriction to calibrate the banding. Sheep were sacrificed acutely (group 1, n=6) and after 1 month of follow-up (group 2, n=6), the reducer was implanted successfully in all animals. It allowed the PA diameter to be reduced from 25 to 10.5 mm. Bare stents were successfully delivered inside the reducer. No paraprosthetic leak was found by injecting contrast dye. After the insertion procedure, signs of intolerance to obstruction were present in all animals and prompted us to dilate the stents from 12 to 16 mm. One animal from group 1 died before a balloon dilatation could be achieved. In the animals from group 2, the mean systolic gradient was 19 and 34.8 mmHg, respectively, at early and late evaluation. CONCLUSION: Implantation of a PA reducer is possible in sheep, through a transcatheter approach allowing intravascular PAB. Miniaturization of the device is necessary to enlarge its use from adulthood to childhood.     

Doppler-guided regulation of a telemetrically operated adjustable pulmonary banding system.

OBJECTIVES: We report on the Doppler-assessed regulation of an adjustable pulmonary artery band (PAB) in an animal model and in our first group of patients. BACKGROUND: Indications for pulmonary artery banding have expanded to include patients requiring a late arterial switch. A telemetry-operated, fully implantable, adjustable PAB system (FloWatch- PAB, Endoart SA, Lausanne, Switzerland) has been developed to facilitate these operations. METHODS: The device was implanted in 13 minipigs (age one to five months, weights 3.2 to 12.0 kg). The main study was performed on nine minipigs with adjustments of the PAB at implantation and at 1, 3, 5, 8, and 12 weeks after, assessed by Doppler pressure gradients. Explanation was performed 12 weeks after surgery. A long-term histology study (6 months and 14 months after surgery) was done on the other four minipigs. After approval by the ethics committee, the device was implanted in eight patients with weights between 2.8 and 9 kg to decrease pulmonary blood flow and pressure and to retrain the left ventricle before arterial switch. The device was progressively tightened, with increasing transband Doppler gradients. Follow-up was one to three months. RESULTS: An excellent correlation between transbanding systolic pressure gradient and degree of PAB constriction was encountered in the minipig study as well as in the human setting. No early or late deaths or reoperations occurred. Malfunction of the device was noted in three of 21 implanted devices. Two were related to surgically inflicted damage at implantation and one to an electronic problem that was fixed by resetting the control device. CONCLUSIONS: The device offers a Doppler-controllable adjustment of pulmonary blood flow. It permits controlled tightening and release of the band, which improves perioperative and postoperative courses and decreases surgical interventions to adjust tightness of the band. It allows a protracted occlusion protocol, which may provide the best effect on retraining the left ventricle.     

Two-stage procedure for pulmonary vascular obstructive disease in Down syndrome with congenital heart disease.

BACKGROUND: Down syndrome patients are characterized by early progression of pulmonary vascular obstructive disease because of insufficient thickness of the pulmonary arterial media. For those with congenital heart disease (CHD) associated with pulmonary hypertension (PH), a 2-stage procedure of pulmonary artery banding (PAB) and then intracardiac repair (ICR) in early infancy is performed to prevent such pulmonary vascular diseases in early infancy. METHODS AND RESULTS: The subjects were 16 patients with Down syndrome who underwent lung biopsy during PAB and ICR. PAB was planned to be performed in early infancy and ICR approximately 1 year later. Efficacy of the 2-stage procedure was retrospectively examined with reference to pulmonary vascular disease and pulmonary diseases. The index of pulmonary vascular disease at PAB fulfilled the indication for ICR, and it was significantly lower at ICR than at PAB (p=0.0469); furthermore, PAB prevented progression of pulmonary diseases. CONCLUSIONS: The results of the lung biopsies support the safety of the 2-stage procedure and show that it is effective for Down syndrome patients with CHD and PH.     

Surgical treatment of ventricular septal defect in the first year of life]

Forty six infants with a large ventricular septal defect (VSD) underwent surgical treatment during the first 12 months of life. Forty three patients ranging in age from 3 to 12 months (mean age 10.4 months) and weighting from 3.0 to 8.2 kg (mean weight 6.8 kg) had primary surgical closure of their VSD. All infants were below the third percentile for weight preoperatively. In 40 patients (93%) the defect was closed through the right atrium. Three patients (7.0%) died in the early postoperative period. Surgically induced heart block occurred in one patient (2.3%). Late results were assessed in 29 surgical survivors (mean follow-up 26 months). There was no late mortality. Relief of congestive heart failure was prompt in all patients following closure of VSD. Right bundle branch block with left anterior hemiblock developed in 5 patients (17.2%), and right bundle branch block alone in 10 patients (34.5%). Three patients (mean age 4.3 months, mean weight 2.7 kg) underwent initial palliative pulmonary artery banding (PAB). There were no operative or late mortality. Closure of VSD and pulmonary artery debanding was performed in two of these patients, with no mortality. Prior to intracardiac correction the pulmonary artery pressure distal to the band was normal; no band related complications were found. Early primary closure is the treatment of choice for symptomatic infants with large VSDs. In particular circumstances, however, PAB may provide effective palliation.     

Surgical outcome of staged univentricular-type repairs for patients with univentricular physiology and pulmonary hypertension

BACKGROUND: There is no consensus about the most appropriate limits of pulmonary artery pressure and vascular resistance in case of patients undergoing univentricular or one and one-half ventricular repair. This study was conducted to analyze the mortality and morbidity of a heterogenous group of patients with a functionally univentricular heart and pulmonary artery hypertension, undergoing pulmonary artery banding followed by univentricular-type repairs. METHODS AND RESULTS: Out of 254 patients undergoing pulmonary artery banding for a functionally univentricular heart with increased pulmonary blood flow, 148 patients underwent definitive second stage surgery. Post-band hemodynamic evaluation revealed persistently high pulmonary artery pressure (> 18 mmHg), and pulmonary vascular resistance (>2.0 Woods units/m2) in 78.3% patients. Sixteen patients with moderate right ventricular hypoplasia were given a one and one-half ventricle repair (Group I), 82 patients a bidirectional Glenn connection (Group II), and 50 patients a fenestrated total cavopulmonary connection (Group III). The overall mortality following second stage surgery for the high pulmonary artery pressure group (n=116) was 30.17%, while none of the low pulmonary artery pressure group died (p=0.0009). Pulmonary hypertensive crises and/or systemic desaturation were the main causes of death at second stage repair. All mortality occurred in patients with mean pulmonary artery pressure > 18 mmHg and pulmonary vascular resistance > 3.5 Woods units/m2. Survivors from this group had persistent morbidity in the form of superior vena caval syndrome and suboptimal oxygen saturation (70-75%). CONCLUSIONS: It is advisable not to proceed with definitive second stage repair if post-pulmonary artery banding mean pulmonary artery pressure is over 25 mmHg and pulmonary vascular resistance exceeds 4.0 Woods units/m2. These patients may possibly be deemed to have undergone definitive palliation during their pulmonary artery banding.     

Effectiveness of Bilateral Pulmonary Artery Banding in Patients with Hypoplastic Left Heart Syndrome and Congenital Heart Defects with A Functional Single Ventricle: A Single-Center Retrospective Study

Background: Bilateral banding of the branches of the pulmonary artery in patients with hypoplastic left heart syndrome (HLHS) and other duct dependent critical neonatal heart malformations can significantly reduce the incidence of severe complications in the postoperative period, especially in severely unstable patients. In our study we compared different surgical techniques of bilateral pulmonary artery banding (PAB) in respect to their success in balancing systemic and pulmonary blood flow. Methods: We included 44 neonates with a HLHS and congenital heart diseases (CHD) with a functional single ventricle underwent a hybrid operation: bilateral PAB and patent ductus arteriosus stenting. The hybrid surgery for method No. 1 is performed as a one-stage procedure, together with patent ductus arteriosus (PDA) stenting. After median sternotomy, two Gore-Tex 1–2 mm wide bands with a diameter of 3–3.5 mm are put. When we apply method No. 2 then the thread is used to create bands. Method No. 3 is distinguished by intraoperative assessment of blood flow at the site of narrowing of the branches of the pulmonary artery and optional stenting of the PDA. The cuff for banding is made of Gore-Tex tubing. Effectiveness when applying method Nos. 1 and 2 is assessed by the change in invasive blood pressure and oxygen saturation after narrowing of the branches of the pulmonary artery. Also, with these techniques PDA stenting by inserting the introducer via pulmonary artery trunk is performed. Results: HLHS with mitral or aortic valve atresia or both was present in 19 patients (43.1%), with severe left heart obstruction resulting in PDA dependent systemic circulation in 16 babies (36.4%). CHD with single ventricle physiology occurred in 9 patients (20.5%). 14 babies (31.8%) undergo the procedure following the method No. 1, 8 patients (18.2%) method No. 2 and 22 patients (50%) method No. 3. Qp/Qs = 1/1 was achieved in 30 patients (30/44, 68.1%): as a result of the method No. 1 was achieved in 5 patients (5/14, 35.7%), method No. 2 in 4 patients (4/8, 50%), method No. 3 in 21 patients (21/22, 95.5%). Multivariate regression analysis revealed that method No. 3 significantly increases the chances of hemodynamic efficacy operations (OR = 35.0; p = 0.005; CI (95%) 3–411.5). Conclusion: Application of the operation technique No. 3 in combination with the intraoperative assessment of blood flow parameters at the site of banding of the branches of the pulmonary artery are the most optimal criteria for achieving Qp/Qs = 1/1. If there are signs of restriction at the level of the foramen ovale, atrioseptostomy should be done in the second stage after bilateral pulmonary banding.     

Mid-Term Outcome of Surgical Treatment in Pediatric Patients with Ebstein’s Anomaly: A Single-Center Cohort Study

Background: Ebstein’s anomaly (EA) is a malformation of the tricuspid valve (TV) and myopathy of the right ventricle (RV). Surgery is now the main treatment for the defect. Objective: To summarize our surgical results and experience based on patients with EA who were under 7 years of age and treated with different surgical treatments. Design: Single-center cohort study of Ebstein’s anomaly. Setting: China. Participants: 80 patients under 7 years old who were diagnosed of EA and underwent different surgical treatments were consecutively enrolled in our research from January 2010 to December 2019. Results: The median age of the 80 patients at the time of surgery was 3.63 years. Sixty-four (80.00%) patients underwent biventricular repair while 13 (16.25%) underwent 1.5-ventricle repair. With the median follow-up 27.50 months, the mid-term survival of the total cohort, 1.5-ventricular repair and biventricular repair was 82.35%, 91.67% and 100%, respectively. The mid-term freedom from reoperation rate was 97.50%, 92.31% and 98.44%, respectively. Mild, moderate and severe TR before surgery occurred in 6 (7.50%), 18 (22.50%) and 56 (70.00%), respectively. The early outcomes of 78 patients were 65 (83.33%), 11 (14.11%) and 2 (2.56%); the mid-term outcomes of 72 patients were 49 (68.06%), 19 (26.38%) and 4 (5.56%). Both early and mid-term valve regurgitations were significantly decreased (p < 0.001) compared with preoperative condition. No more severe regurgitation occurred (p = 0.404), though some early mild regurgitation became acceptable moderate regurgitation during mid-term follow-up (p = 0.036). Conclusion: The overall effect of surgical treatment for EA was good, and most patients could receive biventricular repair at an early stage. The reoperation rate and mid-term mortality were both low and TR was significantly improved.     

Growth of the Pulmonary Valve Annulus after the Modified Blalock-Taussig Shunt in Patients with Tetralogy of Fallot

Background: The surgical outcomes of tetralogy of Fallot (TOF) have evolved dramatically and have resulted in lower mortality rate. Currently, the many cardiac centers have a trend to early single-stage complete repair more than a staged repair. However, the patients who have an early primary repair were required transannular patch augmentation of a pulmonary valve frequently. This effect has been developed a chronic pulmonary insufficiency may lead to right ventricular dilation, dysfunction. In this era, the aim of treatment of TOF is attempted to preserve pulmonary valve annulus for prevent right ventricular dysfunction in the future. The systemic to pulmonary artery shunt is a palliative procedure or known as staged repair for symptomatic patients with TOF. The modified Blalock-Taussig shunt (mBTS) is the most useful systemic to pulmonary shunt and perform as an initial procedure before complete repair. The mBTS can provide increase pulmonary blood flow as well as improve oxygenation and also promote pulmonary artery (PA) growth. However, the effect of this procedure to promote growth of a pulmonary valve annulus is still debate. Objectives: To compare a growth of pulmonary valve annulus between after staged repair and primary repair in patients with TOF (without pulmonary atresia). Methods: A retrospective case-control study, review of patients with TOF underwent total repair at our hospitals from January 2005 and December 2017 was performed, a total number of 112 patients underwent TOF repair. Twenty-nine patients (26%) underwent a staged repair (mBTS group) and 83 (74%) underwent total repair only or primary repair (PR group). We evaluated diameter of pulmonary valve annulus by using echocardiography at the time of first diagnosis and before complete repair on both groups. Results: The age of diagnosis of mBTS group were younger than PR group (p = 0.011). Therefore, pulmonary valve annuls were smaller in mBTS group. (Z-score, −2.93 ± 1.42 vs. −1.89 ± 0.97; p = 0.001). However, the growth potential of pulmonary valve annulus was increase more than PR group significantly (Z-score, −1.46 ± 1.02 vs. −2.11 ± 1.19; p = 0.009) Even though a patent ductus arteriosus was found commonly in PR group (p = 0.018). Conclusions: Our results suggest the systemic to pulmonary shunt or mBTS can promote growth of pulmonary valve annulus in patients with TOF.     

Sustained Reduction in Pulmonary Artery Pressures and Hospitalizations During 2 Years of Ambulatory Monitoring

BackgroundTherapy guided by pulmonary artery (PA) pressure monitoring reduces PA pressures and heart failure hospitalizations (HFH) during the first year, but the durability of efficacy and safety through 2 years is not known.Methods and ResultsThe CardioMEMS Post-Approval Study investigated whether benefit and safety were generalized and sustained. Enrollment at 104 centers in the United States included 1200 patients with NYHA Class III symptoms on recommended HF therapies with prior HFH. Therapy was adjusted toward PA diastolic pressure 8–20 mmHg. Intervention frequency and PA pressure reduction were most intense during first 90 days, with sustained reduction of PA diastolic pressure from baseline 24.7 mmHg to 21.0 at 1 year and 20.8 at 2 years for all patients. Patients completing two year follow-up (n = 710) showed similar 2-year reduction (23.9 to 20.8 mmHg), with reduction in PA mean pressure (33.7 to 29.4 mmHg) in patients with reduced left ventricular ejection. The HFH rate was 1.25 events/patient/year prior to sensor implant, 0.54 at 1 year, and 0.37 at 2 years, with 59% of patients free of HFH during follow-up.ConclusionsReduction in PA pressures and hospitalizations were early and sustained during 2 years of PA pressure-guided management, with no signal of safety concerns regarding the implanted sensor.     

Arterial switch operation after Mustard procedures in adult patients with transposition of the great arteries: is it time to revise our strategy?

Background

After the Mustard or Senning procedure, adults with transposition of the great arteries may have right ventricular failure and require consideration of new therapies. A 2-stage arterial switch operation (ASO) may be performed as an alternative to heart transplantation. This procedure is relatively successful in children, but little is known about the 2-stage ASO in adults. We report our experience in adults undergoing pulmonary arterial banding as the first stage of a planned 2-stage arterial switch procedure after a failed Mustard operation.

Methods and results

Three adult patients with systemic right ventricular failure late after Mustard procedures embarked, through pulmonary artery banding, on a course toward a 2-stage arterial switch at the Toronto General Hospital. Baseline clinical characteristics as well as preoperative hemodynamics were reviewed. Immediate perioperative and postoperative events, hemodynamic measurements, and clinical outcomes were also recorded. Two patients were banded acutely such that their morphologic left ventricular to right ventricular (LV/RV) systolic pressure ratios were >0.65 after the initial banding procedure. The subpulmonary left ventricle failed in both cases. In contrast, the third patient had a more gradual approach to pulmonary artery banding (PAB), with an initial LV/RV pressure ratio of 0.5, which eventually led to a successful conversion to an arterial switch procedure.

Conclusions

Our evidence suggests that in adult patients expected to undergo a 2-stage arterial switch procedure after a failed Mustard operation, acute PAB achieving near-systemic subpulmonary LV pressure leads rapidly to ventricular failure and failure of this treatment strategy. A more gradual approach to PAB may be required to achieve a successful outcome.     

Left ventricular retraining and anatomic correction in teenage patient with congenitally corrected transposition of the great arteries.

Left ventricular (LV) retraining followed by anatomical repair would be a superior alternative in patients with congenitally corrected transposition (ccTGA) having a deconditioned morphologically left ventricle (MLV); however, LV retraining in older children is a challenging task. A retraining process of the MLV in a teenage patient with ccTGA is reported here. Cardiac catheterization at 7 years of age revealed low pressure of the MLV (33/4 mm Hg) and a LV to right ventricular pressure ratio (LVp/RVp ratio) of 0.32. The first pulmonary artery banding (PAB) was performed at 10 years of age. Although the LVp/RVp ratio reached 0.68, there was no evidence of adequate LV hypertrophy. The second PAB was performed 2 years after the initial PAB, resulting in an increase in the LVp/RVp ratio to 0.93 and an adequate LV hypertrophy. The double switch procedure was successfully performed at 13 years of age. Although the ejection fraction of the MLV mildly decreased, the patient has been doing well during a follow-up period of 4 years. The MLV in the teenage patient with ccTGA was successfully trained using a retraining strategy and has sustained systemic circulation after anatomical repair.     

Unilateral pulmonary artery banding to promote contralateral pulmonary artery growth

A full-term baby with double-outlet right ventricle and total anomalous pulmonary venous connection (TAPVC) complicated with left main pulmonary artery (PA) stenosis, presented with heart failure caused by increased pulmonary blood flow. Based on significant discrepancies in size and development between the left and right PAs, we performed right PA banding concomitant with TAPVC repair to promote left PA growth and restrict overall PA flow. PA-graphy performed 3?months after surgery showed marked increase in the left PA size with appropriately low pressure, which enabled us to successfully complete Glenn anastomosis. Under appropriate patient selection, unilateral PA banding for patients with unbalanced peripheral PA size could serve as an effective and less invasive strategy to simultaneously promote PA growth and control PA flow.     

Congenital heart disease: diagnostic approaches, surgical indications and operative results

Our management policies of the main congenital cardiac defects which require emergency surgical intervention in neonate and infancy were reported. Total anomalous pulmonary venous drainage (TAPVD): Recent refinements in two-dimensional (2-D) echocardiography have made it possible to operate for this anomaly using only this modality. When a combination of coarctation is suspected and/or the location of the drainage of the pulmonary vein is obscure, catheterization and angiocardiography are added for detailed diagnosis. It is, however, our policy not to perform right-sided angiocardiography for patients younger than three months of age. Symptomatic cases are operated urgently, although not always on an emergency basis. In our experience, a 12 approximately 24 hour delay with intensive cardiorespiratory and metabolic care may improve the preoperative conditions considerably and increase the chances of a successful surgical repair. Coarctation of the aorta (CoA) and interruption of the aortic arch (IAA): Diagnosis of CoA by echocardiography and aortography by radial artery injection is well established. In neonates and infants with CoA or CoA + patent ductus arteriosus (PDA) and/or ventricular septal defect (VSD), emergency repair of coarctation (usually with subclavian flap aortoplasty) without pulmonary artery banding (PAB) is undertaken on the day of established diagnosis. If a combination of complex cardiac anomalies such as transposition of the great arteries (TGA) and Taussig-Bing anomaly is suspected, catheterization and angiocardiography are added for the detailed diagnosis. Repair of coarctation combined with PAB has been our choice of procedure in these infants. If IAA is suspected, catheterizaton, angiographic and detailed echocardigraphic studies are performed to define the precise anatomy of the aortic arch and associated intracardiac lesions, paying particular attention to the left ventricular outflow tract, as soon as such patients become clinically stable by intensive medical treatment using prostaglandin E1 (PGE1), digitalis and diuretics. Once the diagnosis is established surgical treatment should be carried out without delay. Initial palliation by aortic arch reconstruction with PAB followed by two-stage definitive intracardiac repair have been our choice of procedure in neonates and infants with IAA without severe left ventricular outflow tract obstruction.(ABSTRACT TRUNCATED AT 400 WORDS)     

Remote control of pulmonary blood flow: a dream comes true

The indication for pulmonary artery banding is currently limited by several factors. Previous attempts have failed to produce adjustable pulmonary artery banding with reliable external regulation. An implantable, telemetrically controlled, battery-free device (FloWatch) developed by EndoArt SA, a medical company established in Lausanne, Switzerland, for externally adjustable pulmonary artery banding was evaluated on minipigs and proved to be effective for up to 6 months. The first human implant was performed on a girl with complete atrioventricular septal defect with unbalanced ventricles, large patent ductus arteriosus and pulmonary hypertension. At one month of age she underwent closure of the patent ductus arteriosus and FloWatch implantation around the pulmonary artery through conventional left thoracotomy. The surgical procedure was rapid and uneventful. During the entire postoperative period bedside adjustments (narrowing or release of pulmonary artery banding with echocardiographic assessment) were repeatedly required to maintain an adequate pressure gradient. The early clinical results demonstrated the clinical benefits of unlimited external telemetric adjustments. The next step will be a multi-centre clinical trial to confirm the early results and adapt therapeutic strategies to this promising technology.     

Pulmonary Hemodynamics and Outcome in a Large Cohort of Patients with Sinus Venosus Septal Defect

Background: Left-to-right shunt in sinus venosus septal defect (SVSD) may affect resistive (pulmonary vascular resistance–PVR) and elastic (pulmonary artery compliance-PAC) pulmonary artery properties. This study aimed at evaluating (1) impact of age, (2) pulmonary hemodynamics, and (3) outcome in a large cohort of SVSD patients. Methods: This study included 136 patients with SVSD (median age at diagnosis 14 (IQR 5–48) years, 47% male) of which 87 underwent catheterization. Pressures were measured and cardiac output was evaluated using the Fick principle at diagnosis. PVR, PAC and their product (RC time) were calculated. Results: Surgical repair was performed in 128 (94%) at a median age of 13 (IQR 5– 43) years. During a median follow-up time of 31 (IQR 17–55) years, 12 (9%) patients died, 13 (10%) developed heart failure, 4 (3%) Eisenmenger syndrome, 19 (14%) atrial arrhythmia, 6 (4%) sick sinus syndrome and 7 (5%) required pacemaker implantation. In those who underwent catheterization, median shunt ratio was 2.5 (IQR 2.0–2.9). Thirty (34%) had mean PA pressure ≥25 mmHg. PVR indexed, PAC indexed, and RC time was 3.5 (IQR 2.4–7.5) WU.m², 1.8 (IQR 1.3–2.5) mL/mmHg.m² and 0.39 (0.26–0.53) sec with an inverse hyperbolic relationship between PVR and PAC. Mean PA pressure (P < 0.0001); wedge pressure (P = 0.001), PVR indexed (P = 0.002) and PAC indexed (P = 0.002) changed significantly with age at diagnosis, but shunt ratio did not. Conclusion: SVSD has good long-term outcome, albeit with late morbidities. Thirty-four percent has mean PA pressure ≥25 mmHg, but Eisenmenger syndrome is rare (3%). PVR and PAC are inversely related and change significantly with older age.     

Remote invasive monitoring of pulmonary artery pressures in heart failure patients: Initial experience in Portugal in the context of the Covid-19 pandemic

BackgroundDecompensated heart failure (HF) is associated with poor short- and long-term prognosis. Remote invasive monitoring of pulmonary artery pressures (PAP) enables early detection of HF decompensation before symptoms occur and may improve clinical outcomes. We aimed to describe our initial experience with the use of the CardioMEMS? remote monitoring system in patients with HF, including its safety and effectiveness.Methods and resultsFive patients with HF in New York Heart Association class III and at least one hospitalization due to decompensated HF in last 12 months, who underwent invasive remote monitoring of PAP, were included in this prospective registry. The median age was 66.0 years (interquartile range [IQR] 50.5-77.5 years), 80.0% were men and all had HF with reduced ejection fraction. The pulmonary artery (PA) sensor was placed in a left PA branch in all patients and no major procedural complications occurred. In median follow-up of 40 days (IQR 40-61 days), a total of 271 pressure readings were transmitted, patient compliance was 100% and freedom from sensor failure 98.1%. In three patients, PAP remained within the goal during follow-up. Two patients presented an increase in PAP to values above the targets, despite the absence of symptom worsening. These required dietary and diuretic dose adjustment, without the need for outpatient clinic visits, which reduced PAP. No hospitalizations for HF or deaths occurred during follow-up.ConclusionHemodynamic-guided HF monitoring was safe and effective and may be a useful adjunctive tool to the standard-of-care management in selected HF patients, particularly in the context of the COVID-19 pandemic, where a reduction in the number of health care visits may be desirable.     

Diagnostic value of parasternal pulmonary artery short-axis view for the anomalous origin of the left coronary artery from the pulmonary artery

ObjectiveTo evaluate the diagnostic value of parasternal pulmonary artery (PA) short-axis view for the anomalous origin of left coronary artery (LCA) from the pulmonary artery by echocardiography.MethodsA total of 13 patients (3 boys) aged from 2 months to 12 years were enrolled. Transthoracic echocardiography, including cross-sectional imaging and color Doppler flow imaging, were performed, and their diagnoses were confirmed by operation.ResultsAmong the 13 patients, 7 had LCA originated from the left posterior wall of PA, 2 from the posterior wall, and 4 from the right posterior wall. The PA short-axis view could visualize the anomalous origin of the LCA from left posterior or posterior wall of PA clearly. The LCA and aortic wall were overlapping at 3–4 o’clock at PA short-axis view in the patients with LCA originating from the right posterior wall of PA. It was similar with the image of the LCA originating from the aorta. But the blood flow was opposite to that of LCA with normal origin.ConclusionsThe parasternal PA short-axis view is a good view to visualize the anomalous origin of the LCA.     

Pulmonary artery banding

Pulmonary artery banding is a palliative surgical procedure used to treat functionally univentricular hearts, multiple ventricular septal defects and complete atrioventricular septal defects. Pulmonary artery banding has recently gained in interest for left ventricular retraining and hypoplastic left heart malformations. The indication for pulmonary artery banding is limited by several factors: difficulty of determining optimal band tightness, influence of perioperative variables with mutual interference, agerelated variability of the ventricular adaptive response, difficulty of securing sufficiently tight banding in older children, repeated surgical procedures needed to adjust the band perimeter, long periods under intensive respiratory and/or pharmacological support, and the frequent need for reconstruction of the pulmonary artery at intracardiac repair. The need for adjustable banding has now been met by the clinical availability of telemetrically controlled adjustable pulmonary artery banding (FloWatch, EndoArt, Lausanne, Switzerland), which has been successfully tested in clinical practice after favourable evaluation in animal experiments. This new wireless, battery free, implantable device (FloWatch), allows repeated progressive occlusion and reopening of the device by remote control, at the desired percentage occlusion, without the need for reoperation to adjust the band. The introduction of FloWatch has brought substantial changes in the management of patients with increased pulmonary artery blood flow and pressure, a reduction in the mortality and morbidity associated with conventional banding, and significant shortening of intensive care unit and hospital stay. New therapeutic strategies could well extend the applicability of this device in patients with congenital heart defects.