Partial anomalous pulmonary venous connection to superior vena cava that overrides across the intact atrial septum and has bi-atrial connection in a 75-year-old female presenting with pulmonary hypertension

One of many causes of right heart failure (RHF) is partial anomalous pulmonary venous connection (PAPVC). We present a rare entity of isolated supra-cardiac PAPVC, as right pulmonary vein drains into the superior vena cava (SVC) with intact atrial septum, precipitating RHF.A 55-year-old man with hypertension, diabetes mellitus, coronary artery disease, presenting with syncope. On examination blood pressure was 90/44 mm Hg and heart rate of 44 bpm, lungs were clear on auscultation, jugular venous distension was present, prominent S2 heart sound, and bilateral pitting edema of the lower extremities. Laboratory studies were significant for brain natriuretic peptide (BNP) of 504 pg/mL, troponin I of 0.06 ng/mL, and glycated hemoglobin (HgA1c) of 11.9%. Electrocardiography was significant for left atrial dilation and right ventricular hypertrophy. Transthoracic echocardiography (TTE) showed severe right ventricular dilation, left ventricular hypertrophy, and severe tricuspic regurgitation with pulmonary artery systolic pressure of 85 mm Hg. Additionally, on the TTE ejection fraction was noted to be 55% with no evidence of atrial septal defect (ASD). Cardiac catheterization and computed tomography angiogram (CTA) revealed severe pulmonary hypertension and drainage of the right pulmonary vein into the superior vena cava.We have described a case of an isolated supra-cardiac variant of right pulmonary vein draining into the SVC. ASD is absent in isolated form of PAPVC, our case demonstrated an intact atrial septum in a supra-cardiac variant. Studies have indicated that 82% of patients with PAPVC have an ASD, distinguishing our case as an uncommon entity.Patient was discharged with optimized doses of bumetanide, metoprolol, and was offered surgery for the definitive treatment. Surgical prognosis is excellent and the perioperative mortality rate is less than 0.1%.Abstract ID: 5 Figure 1Cardiac catheterization illustrating drainage from the right pulmonary vein anomalously into the superior vena cava.

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Surgical Treatment of Partial Anomalous Pulmonary Venous Return into the Superior Vena Cava with Special Reference to a Case with Intact Atrial Septum

The Tohoku Journal of Experimental Medicine ◽

10.1620/tjem.92.221 ◽

1967 ◽

Vol 92 (3) ◽

pp. 221-228

Author(s):

Togo Horiuchi ◽

Tadaaki Abe ◽

Toshiro Matsumoto ◽

Takeshi Ishitoya

Keyword(s):

Surgical Treatment ◽

Special Reference ◽

Superior Vena Cava ◽

Venous Return ◽

Superior Vena ◽

Vena Cava ◽

Atrial Septum ◽

Intact Atrial Septum ◽

Anomalous Pulmonary Venous Return

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Partial Anomalous Pulmonary Venous Connection: Forty-Six Years of Follow-Up

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135120960482 ◽

2021 ◽

Vol 12 (1) ◽

pp. 70-75

Author(s):

Anne Kathrine M. Nielsen ◽

Vibeke E. Hjortdal

Keyword(s):

Superior Vena Cava ◽

Superior Vena ◽

Sinus Node ◽

Vena Cava ◽

Node Disease ◽

Sinus Node Disease ◽

Patch Technique ◽

Anomalous Pulmonary Venous Connection

Background: Surgical repair of partial anomalous pulmonary venous connection (PAPVC) may disturb the electrical conduction in the atria. This study documents long-term outcomes, including the late occurrence of atrial tachyarrhythmia and bradyarrhythmia. Methods: This retrospective study covers all PAPVC operations at Aarhus University Hospital between 1970 and 2010. Outcome measures were arrhythmias, sinus node disease, pacemaker implantation, pathway stenosis (pulmonary vein(s), intra-atrial pathway, and/or superior vena cava), and mortality. Data were collected from databases, surgical protocols, and hospital records until May 2018. Results: A total of 83 patients were included with a postoperative follow-up period up to 46 years. Average age at follow-up was 43 ± 21 years. During follow-up, new-onset atrial fibrillation or atrial flutter appeared in four patients (5%). Sinus node disease was present in nine patients (11%). A permanent pacemaker was implanted in seven patients (8%) at an average of 12.7 years after surgery. Pulmonary venous and/or superior vena cava obstruction was seen in five patients (6%). Stenosis was most prevalent in the two-patch technique, and arrhythmia was most prevalent in the single-patch technique. Sixty-seven (81%) of 83 patients had neither bradyarrhythmias nor tachyarrhythmias or pacemaker need. Conclusions: This study contributes important long-term data concerning the course of patients who have undergone repair of PAPVC. It confirms that PAPVC can be operated with low postoperative morbidity. However, late-onset stenosis, bradyarrhythmias and tachyarrhythmias, and need for pacemaker call for continued follow-up.

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Rare Finding of Partial Anomalous Pulmonary Venous Connection With an Intact Atrial Septum Presenting as Right Heart Failure and Pulmonary Hypertension in an Adult

CHEST Journal ◽

10.1378/chest.1118687 ◽

2011 ◽

Vol 140 (4) ◽

pp. 96A

Author(s):

Richie Rana ◽

David Padua ◽

Rick Tennant ◽

Greg Bierer ◽

Jeanne Wallace ◽

...

Keyword(s):

Heart Failure ◽

Pulmonary Hypertension ◽

Right Heart Failure ◽

Atrial Septum ◽

Right Heart ◽

Rare Finding ◽

Intact Atrial Septum ◽

Anomalous Pulmonary Venous Connection

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Superior vena cava varix incriminating obstructive total anomalous pulmonary venous connection in a 42-day-old infant

International Journal of Cardiology ◽

10.1016/s0167-5273(01)00594-0 ◽

2002 ◽

Vol 82 (1) ◽

pp. 87-90

Author(s):

Meng-Luen Lee ◽

Shyh-Jye Chen ◽

Ing-Sh Chiu

Keyword(s):

Superior Vena Cava ◽

Superior Vena ◽

Vena Cava ◽

Anomalous Pulmonary Venous Connection

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An Alternative Approach for a High Partially Anomalous Pulmonary Venous Connection to the Superior Vena Cava: “A Drawbridge Technique”

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/21501351211070289 ◽

2022 ◽

pp. 215013512110702

Author(s):

Roman Sekelyk ◽

Dmytro Kozhokar ◽

Vsevolod Safonov ◽

Illya Yemets

Keyword(s):

Superior Vena Cava ◽

Superior Vena ◽

Vena Cava ◽

Alternative Technique ◽

Alternative Approach ◽

Anomalous Pulmonary Venous Connection

We present an alternative technique for the repair of a high partially anomalous pulmonary venous connection to the superior vena cava.

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Cor Triatriatum in Association With a Unique Form of Partial Anomalous Pulmonary Venous Connection

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/21501351211046907 ◽

2021 ◽

pp. 215013512110469

Author(s):

Lou Capecci ◽

Richard D. Mainwaring ◽

Inger Olson ◽

Frank L. Hanley

Keyword(s):

Superior Vena Cava ◽

Superior Vena ◽

Pulmonary Veins ◽

Vena Cava ◽

Venous Drainage ◽

Cor Triatriatum ◽

Unique Form ◽

Elective Repair ◽

The Right ◽

Anomalous Pulmonary Venous Connection

Cor triatriatum may be associated with abnormalities of pulmonary venous anatomy. This case report describes a unique form of partial anomalous pulmonary venous connection. The patient presented at 5 weeks of age with symptoms of tachypnea and poor feeding. Echocardiography demonstrated cor triatriatum and partial anomalous pulmonary venous drainage of the right upper lung. The patient underwent urgent repair of cor triatriatum. It was elected to not address the partial anomalous pulmonary venous connection at that time. The patient returned at age 19 months for elective repair of the anomalous pulmonary venous connection. There was also a large vein connecting the right lower pulmonary veins to the superior vena cava. This was repaired by dividing the superior vena cava along a vertical axis to redirect the flow of the anomalous pulmonary veins through the connecting vein to the left atrium. This report describes the anatomy and surgical approach to a unique form of anomalous pulmonary venous connection.

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Orthodeoxia without Platypnea from Interatrial Defect Associated with Persistent Left Superior Vena cava in the Absence of Pulmonary Hypertension

Respiration ◽

10.1159/000070070 ◽

2003 ◽

Vol 70 (2) ◽

pp. 207-210 ◽

Cited By ~ 1

Author(s):

Mauro Maniscalco ◽

Giovanni Dialetto ◽

Giovanni Tufano ◽

Annamaria Romano ◽

Matteo Sofia

Keyword(s):

Pulmonary Hypertension ◽

Superior Vena Cava ◽

Superior Vena ◽

Vena Cava ◽

Left Superior Vena Cava ◽

Persistent Left Superior Vena

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Interatrial shunt for chronic pulmonary hypertension: differential impact of low-flow vs. high-flow shunting

AJP Heart and Circulatory Physiology ◽

10.1152/ajpheart.00496.2008 ◽

2009 ◽

Vol 296 (3) ◽

pp. H639-H644 ◽

Cited By ~ 17

Author(s):

Andreas Zierer ◽

Spencer J. Melby ◽

Rochus K. Voeller ◽

Marc R. Moon

Keyword(s):

Pulmonary Hypertension ◽

Cardiac Output ◽

Superior Vena Cava ◽

Diastolic Pressure ◽

Superior Vena ◽

Vena Cava ◽

High Flow ◽

Low Flow ◽

Right Atrial ◽

Chronic Pulmonary Hypertension

The purpose of the present study was to determine for the first time the qualitative and quantitative impact of varying degrees of interatrial shunting on right heart dynamics and systemic perfusion in subjects with chronic pulmonary hypertension (CPH). Eight dogs underwent 3 mo of progressive pulmonary artery banding, following which right atrial and ventricular end-systolic and end-diastolic pressure-volume relations were calculated using conductance catheters. An 8-mm shunt prosthesis was inserted between the superior vena cava and left atrium, yielding a controlled model of atrial septostomy. Data were obtained 1) preshunt or “CPH”; 2) “Low-Flow” shunt; and 3) “High-Flow” shunt (occluding superior vena cava forcing all flow through the shunt). With progressive shunting, right ventricular pressure fell from 72 ± 19 mmHg (CPH) to 54 ± 17 mmHg (Low-Flow) and 47 ± 17 mmHg (High-Flow) ( P < 0.001). Cardiac output increased from 1.5 ± 0.3 l/min at CPH to 1.8 ± 0.4 l/min at Low-Flow (286 ± 105 ml/min, 15% of cardiac output; P < 0.001), but returned to 1.6 ± 0.3 l/min at High-Flow (466 ± 172 ml/min, 29% of cardiac output; P = 0.008 vs. Low-Flow, P = 0.21 vs. CPH). There was a modest rise in systemic oxygen delivery from 252 ± 46 ml/min at CPH to 276 ± 50 ml/min at Low-Flow ( P = 0.07), but substantial fall to 222 ± 50 ml/min at High-Flow ( P = 0.005 vs. CPH, P < 0.001 vs. Low-Flow). With progressive shunting, bichamber contractility did not change ( P = 0.98), but the slope of the right atrial end-diastolic pressure volume relation decreased ( P < 0.04), consistent with improved compliance. This study demonstrated that Low-Flow interatrial shunting consistently improved right atrial mechanics and systemic perfusion in subjects with CPH, while High-Flow exceeded an “ideal shunt fraction”.

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