scholarly journals Noonan syndrome – then and now

1999 ◽  
Vol 9 (6) ◽  
pp. 545-546 ◽  
Author(s):  
Jacqueline Noonan

The report by Danetz, Donofrio and Embrey1 provides an interesting long term follow-up on the first patient diagnosed by me with what is now known as Noonan syndrome. In 1960, when this patient was seen, cardiac ultrasound was unavailable and left-sided cardiac catheterization uncommon. He had severe pulmonary stenosis, and did well following surgery. Unfortunately, his follow-up was not adequate and left-sided obstruction was not recognized until he became symptomatic some thirty years later. Early reports stressed the dysplastic pulmonary valve, with pulmonary stenosis as a common finding.2 By 1972, the report of Ehlers et al.3 brought attention to the left-sided involvement in the Turner phenotype.

Author(s):  
Simone Ghiselli ◽  
Cristina Carro ◽  
Nicola Uricchio ◽  
Giuseppe Annoni ◽  
Stefano M Marianeschi

Abstract OBJECTIVES Chronic pulmonary valve (PV) regurgitation is a common late sequela after repair of congenital heart diseases like tetralogy of Fallot or pulmonary stenosis, leading to right ventricular dilatation and failure and increased late morbidity and mortality. Timely reoperation may lead to a complete right ventricular recovery. An injectable PV allows pulmonary valve replacement, with or without cardiopulmonary bypass, under direct observation, thereby minimizing the impact of surgery on cardiac function. The aim of this study was to evaluate the feasibility and mid- to long-term clinical outcomes with this device. METHODS From April 2007 to October 2019, a total of 85 symptomatic patients with severe pulmonary regurgitation or pulmonary stenosis underwent pulmonary valve replacement with an injectable stented pulmonary prosthesis. Data were collected from the international proctoring registry. Mean patient age was 26.7 years. The underlying diagnosis was repaired tetralogy of Fallot in 69.4% patients; moderate or severe pulmonary regurgitation was present in 72.9%. All patients had echocardiographic scans before the operation and during the follow-up period. A total of 54.1% patients also had preoperative/postoperative cardiac magnetic resonance imaging (MRI) or catheterization; 25.9% had off-pump implants. In 53% patients, pulmonary valve replacement was associated with the repair of other cardiac defects. RESULTS Minor postoperative complications were observed in 10.8% patients. The overall mortality rate was 2.3%; mortality after valve replacement was linked to a severe cardiac insufficiency and it was not related to a prosthesis failure; 1 prosthesis was explanted from 1 patient because of endocarditis, and 6% of patients developed PV stenosis; minor complications occurred in 4.8%. The mean follow-up period was 4.8 years (2 months–12.7 years); 42% of the patients were followed for more than 5 years. Follow-up echocardiography and cardiac MRI showed a significant reduction in RV size and low gradients across the PV. CONCLUSIONS An injectable PV may be implanted without cardiopulmonary bypass and in a hybrid operating theatre with minimal surgical impact. The bioprosthesis, available up to large sizes, has a low profile, laminar flow and no risk of coronary artery compression. Incidence of endocarditis is rare. The lack of a suture ring permits the implant of a relatively larger prosthesis, thereby avoiding a right ventricular outflow tract obstruction. This device permits future percutaneous valve-in-valve procedures, if needed. Results concerning durability are encouraging, and mid- to long-term haemodynamic performance is excellent.


2018 ◽  
Vol 11 (2) ◽  
pp. 196-199 ◽  
Author(s):  
Ruben Mühl-Benninghaus ◽  
Alena Haußmann ◽  
Andreas Simgen ◽  
Toshiki Tomori ◽  
Wolfgang Reith ◽  
...  

Background and purposeIn recent years, implantation of flow diverters has emerged as an option for the endovascular treatment of intracranial aneurysms. One complication of treatment with flow diverters is the occurrence of in-stent stenosis, which has been reported to be partially reversible. The purpose of our study was to assess the incidence and dynamics of in-stent stenosis on angiographic short term and long term follow-up after treatment with flow diverters.MethodsA retrospective review of our prospectively maintained database identified all patients with intracranial internal carotid artery (ICA) aneurysms treated by flow diverters at our institution between 2014 and 2017. Clinical charts, procedural data, and angiographic results were reviewed.Results18 patients were included. The mean short term follow-up was 92±19 days and mean long term follow-up was 449±120 days after treatment. No neurologic complications were observed. There was no procedure related mortality. Long term angiographic results showed complete occlusion in 83.3%, neck remnants in 11.1%, and incomplete occlusion in 5.5% of cases. In-stent stenosis was observed in all cases. Mean stenosis improved significantly from 30% on short term follow-up to 12% on long-term follow-up (P<0.0001).ConclusionIn-stent stenosis is a common finding on short term follow-up after the treatment with flow diverters but improves over time.


2006 ◽  
Vol 92 (2) ◽  
pp. 128-132 ◽  
Author(s):  
A C Shaw ◽  
K Kalidas ◽  
A H Crosby ◽  
S Jeffery ◽  
M A Patton

2016 ◽  
Vol 102 (1) ◽  
pp. 186-191
Author(s):  
Tai Fuchigami ◽  
Mitsugi Nagashima ◽  
Takeshi Hiramatsu ◽  
Goki Matsumura ◽  
Minori Tateishi ◽  
...  

2019 ◽  
Vol 10 (5) ◽  
pp. 543-551 ◽  
Author(s):  
Pasangi Madhuka Wijayarathne ◽  
Peter Skillington ◽  
Samuel Menahem ◽  
Amalan Thuraisingam ◽  
Marco Larobina ◽  
...  

Background: Following corrective surgery in infancy/childhood for tetralogy of Fallot (TOF) or its variants, patients may eventually require pulmonary valve replacement (PVR). Debate remains over which valve is best. We compared outcomes of the Medtronic Freestyle valve with that of the pulmonary allograft valve following PVR. Methods: A retrospective study was undertaken from a single surgical practice of adult patients undergoing elective PVR between April 1993 and March 2017. The choice of valve was at the surgeon’s discretion. There was a trend toward the almost exclusive use of the more readily available Medtronic Freestyle valve since 2008. Results: One hundred fifty consecutive patients undergoing 152 elective PVRs were reviewed. Their mean age was 33.8 years. Ninety-four patients had a Medtronic Freestyle valve, while 58 had a pulmonary allograft valve. There were no operative or 30-day mortality. The freedom from reintervention at 5 and 10 years was 98% and 98% for the pulmonary allograft and 99% and 89% for the Medtronic Freestyle. There was no significant difference in the rate of reintervention, though this was colored by higher pulmonary gradients across the Medtronic Freestyle despite its shorter follow-up. Conclusions: Pulmonary valve replacement following previous surgical repair of TOF or its variants was found to be safe with no significant differences in mortality or reintervention between either valve. Although the Medtronic Freestyle valve had a greater tendency toward pulmonary stenosis, additional follow-up is needed to further document its long-term outcomes.


Medicina ◽  
2019 ◽  
Vol 55 (9) ◽  
pp. 608 ◽  
Author(s):  
Liekiene ◽  
Bezuska ◽  
Semeniene ◽  
Cypiene ◽  
Lebetkevicius ◽  
...  

Background and Objectives: Infective endocarditis in the pulmonary position is a rare disease. Isolated pulmonary valve endocarditis is extremely rare. The aim of our study was to assess patients who were treated surgically for pulmonary endocarditis at our institution from January 2003 to December 2017. Materials and Methods: We analyze eight cases of infectious endocarditis in pulmonary position out of 293 patients who were operated for infective endocarditis (2.7%, 8/293). Only two of these eight patients were not related to congenital heart malformation. They were followed for early and late mortality, long-term survival, postoperative morbidity and reoperations. Results: Among six patients suffering from congenital heart disease, four patients underwent corrections of pulmonary valve malformation previously, and their infected grafts were replaced by two allografts and two xenografts. The two other patients had replaced their infected pulmonary valves with allografts. Two non-congenital patients with pulmonary valve endocarditis underwent valve replacement with biological prosthesis. All patients survived the early postoperative course. The mean follow-up time was 9.1 (interquartile range (IQR), 5.3–12.6) years. The long-term follow-up included seven patients. One patient (12.5%, 1/8) died more than 4 years after the surgery due to sepsis. Pulmonary endocarditis was the rarest endocarditis treated surgically (p < 0.001). Conclusion: Surgery for infective endocarditis in the pulmonary position (IEPP) is an effective method of treatment with excellent early outcome and good late results despite a very uncommon pathology and few operations being performed. Surgery performed earlier may make the procedure less radical.


Sign in / Sign up

Export Citation Format

Share Document