Page 60 - Journal of Structural Heart Disease Volume 5, Issue 4

Meeting Abstracts
122
Cardiac catheterization in early infancy demonstrated suprasystemic right ventricular pressure and severe branch pulmonary artery stenosis. Due to heavy calcification, angioplasty was not attempted and she was followed con- servatively. At clinical follow up at 3 1⁄2 years old, her par- ents reported that she was easily short of breath with any activity. Her echocardiogram showed a severely dilated right ventricle with depressed systolic function. Due to the risk of further right ventricular failure and potential life-threatening arrhythmias, she was referred for percu- taneous evaluation and possible intervention. ECMO sup- port was on standby, and a cardiothoracic surgeon was available to intervene emergently if necessary. At cath- eterization, right ventricular systolic pressure was 180% systemic. Heavy calcification was seen in the proximal segmental branches to both lungs. The proximal left lower lobe pulmonary artery branch narrowed dramatically to 1.3 to 2 mm. The vessel was cannulated with a 0.014” wire and serial dilation was performed first with a 4 mm cutting balloon and then a 5 mm cutting balloon. Angioplasty of the lingular branch was then performed with a 2.5 mm cor- onary balloon. Repeat angiography demonstrated relief of left-sided obstruction without any extravasation of con- trast. There was a thread-like connection to the right lower lobe that measured approximately 1.3 mm in diameter. This was cannulated with one 0.014” wire, and a separate 0.014” wire was positioned in the anterior segment of the lower lobe. Balloon angioplasty was performed along the length of the narrowed vessel with a 4 mm cutting balloon. Final angiography showed relief of obstruction bilaterally without contrast extravasation. Pressure measurements showed that right ventricular pressure was reduced from 180% systemic to 80% systemic at the end of the case. The patient was admitted to the ICU to monitor for reperfusion injury and was discharged home after two days without incident. After five months in clinical outpatient follow-up, parents reported a sustained increase in her activity level and endurance.
Discussion: Our case is the first to demonstrate that a percutaneous approach can be safely and effectively employed for the rare patient with calcified pulmonary artery stenosis after twin-to-twin transfusion syndrome. Previous reports have only demonstrated relief of obstruc- tion with surgical arterioplasty. Even with heavy calcifica- tion, a percutaneous approach could be considered as an alternative to surgery in patients with a poor prognosis and failing right ventricle.
Marco Ruiz-Ontiveros, Alejandro Flores-Arizmendi, Antonio Salgado-Sandoval
National Medical Center "20 De Novembre", Mexico City, Mexico
Hereditary hemorrhagic telangiectasia or Osler Weber Rendu syndrome is an autosomal dominant multiorgan disorder caused by a genetic defect in the protein trans- forming growth factor, resulting in a fibrovascular dysplasia with multiple arteriovenous malformations accompanied by telangiectasia also present with symptoms such as cya- nosis, dyspnea, high output heart failure, hemoptysis and paradoxical embolization. Pulmonary arteriovenous fistula (PAVF) is abnormally dilated vessels that provide a right-to- left shunt between pulmonary artery and pulmonary vein. The fistula itself is typically a thin-walled aneurysmal vas- cular structure that tends to increase over time.
Case Report: A 9-year-old girl was referred to our insti- tution due to chronic cyanosis, dizziness and headache during the last month. Her family history was negative for cardiac diseases. On physical examination, peripheral cyanosis (oxygen saturation: 70%). She had no murmurs. EKG showed cardiac axis to the right (150˚). An echocardio- gram reported heart without structural anomalies. CT scan reported a PAVF in the lower left lobe, a pulmonary seques- tration of 8.4mm. The interventional catheterization was performed to occlude the fistula. Under general endotra- cheal anesthesia, the right femoral vein was cannulated. A 7 Fr Mullins sheath was positioned over a exchange guide- wire in the left lower lobal artery. With the aid of a Head Hunter catheter the vessels were opacified with contrast injections. We start with the central one, using the same sheath a 10mm Amplatzer Vascular Plug was advanced and exteriorized, After confirmation of proper positioning, the device was released. The second one that was near for few millimeters, it was opacificied and after measured a 12mm Amplatzer Vascular Plug was advanced and released after confirmation of proper positioning. This process was repeated with other two PAVFs, both close to the previous, using 6mm and 8mm Amplatzer Vascular Plug devices. A final angiogram demonstrated complete occlusion of the four vessels . The peripheral oxygen saturation on room air immediately increased from 70% to 95%
After 6 years the patient was asymptomatic, but the oxy- gen saturation decreased to 82%, so it was decided to per- form a new diagnostic and interventional catheterization. Under general endotracheal anesthesia, the right femoral vein was cannulated. A 6Fr sheath was positioned over a exchange guidewire in the left lower lobal artery. With the aid of a 6Fr Judkin right catheter the vessel were opacified with contrast injections. It was a unique and large vessel.
79. OCCLUSION OF MULTIPLE ARTERIOVENOUS FISTULAS IN A GIRL WITH HEREDITARY HEMORRHAGIC TELANGIECTASIA
PULMONARY
Journal of Structural Heart Disease, August 2019
Volume 5, Issue 4:75-205